A SHORT TERM STUDY ON PELVIURETERIC JUNCTION

OBSTRUCTION IN CHILDREN

Revised and Resubmission of Dissertation For the final examination of

MCh Branch V (Paediatric Surgery) August 2006

The Tamilnadu Dr.M.G.R.Medical University, Chennai.

CERTIFICATE

This is to certify that this is the bonafide dissertation work done by

Dr.A.RAVIKUMAR, MS., submitted for the MCh Paediatric surgery

examination held on August 2006 under the title of “A SHORT TERM STUDY

ON PELVIURETERIC JUNCTION OBSTRUCTION IN CHILDREN” under my

guidance and supervision.

Prof. Dr.RAMESH RATHINAM, MS., MCh Professor and Head, Department of Peadiatric Surgery, Madurai Medical College, Madurai.

ACKNOWLEDGEMENT

I take this opportunity to express my sincere gratitude to my unit chief

Prof. Dr.T. GNANASEELAN MS., MCh. Professor and Head of the

department of Paediatric surgery, Madurai Medical College, Madurai for his

constant guidance and encouragement throughout the period of this study and

without whose help, this study would not have been possible.

I am very much thankful to Prof. Dr. Ramesh Rathinam MS, MCh for

extending his help to conduct this study.

I am very much thankful to THE DEAN, Madurai Medical College for

permitting me to do the study and extending all help to conduct this study.

I thank our Assistant professors Dr.A.Athigaman M.S, MCh.,

Dr.A.KachiMohideen M.S,MCh., Dr.S.Ramamoorthy M.S, MCh.,

Dr.B.Hemanth kumar M.S, MCh for their valuable guidance given during my

study.

The study would not have been completed, but for the cooperation of our

patients. I extend my thanks to them.

CONTENTS 1. INTRODUCTION 1 2. MATERIALS AND METHODS 2 3. AIM OF THE STUDY 3 4. REVIEW OF LITERATURE 4

EMBRYOLOGY OF PELVIURETERIC JUNCTION

PHYSIOLOGY PATHOPHYSIOLOGY ETIOLOGY

5. DIAGNOSTIC WORKUP 17 6. TREATMENT 38 7. RESULTS OF STUDY AND DISCUSSION 89 8. CONCLUSION 96 9. PROFORMA 10. MASTER CHART 11. BIBLIOGRAPHY

INTRODUCTION

Hydronephrosis is defined as dilatation of the renal collecting system as a

result of either inadequate drainage or retrograde flow of urine. Pelviureteric

junction obstruction (PUJ stenosis) represents 44% of all postnatal causes of

hydronephrosis.

Congenital hydronephrosis, caused by pelviureteric junction obstruction,

has challenged the ingenuity of paediatric surgeons and remains one of the most

enigmatic clinical problems today. Historically, pain, infection, stone and

hematuria have been the classic and compelling indications for intervention.

Currently with the increased widespread use of high-resolution real time

fetal ultrasonography, antenatal hydronephrosis with minor dilatation of the upper

urinary tract is being detected with increased frequency.

The challenge for today’s Paediatric surgeons in the management of

hydronephrosis is to decide which patients can be observed, which can be

medically managed and which require surgery.

MATERIALS AND METHODS

60 cases of hydronephrosis were studied in the department of Paediatric

surgery in Madurai medical college, Madurai during the period from July 2003 to

September 2005. Out of 60, 41 had Pelviureteric junction obstruction.

All cases were evaluated by recording antenatal history, age, sex,

presenting symptoms, signs and if there is any previous treatment.

All the cases were investigated with urine culture, blood urea, creatinine

estimation, ultrasonogram abdomen and intravenous urogram. Some were

subjected to CT scan abdomen with contrast and Diuretic renogram.

The conventional open Anderson Hynes pyeloplasty was done in 39 cases.

All the patients were followed regularly with ultrasonogram and few of them with

intravenous pyelogram and diuretic renogram.

AIM OF THE STUDY

1. To analyse the incidence of pelviureteric junction obstruction

2. To analyse the clinical presentation

3. To analyse the various modalities of treatment

4. To analyse the pre and postoperative function of the affected kidney.

REVIEW OF LITERATURE

EMBRYOLOGY

At around the 5th week of intrauterine life, the ureteral bud develops as a

diverticulum from the caudal segment of the mesonephric duct near its entry into

cloaca. The ureteral bud grows cephalad and penetrates the metanephric blastema

resulting tissue induction of the undifferentiating mesenchyme and transforming it

into the functioning nephrons of the metanephric kidney.

The ureteric bud undergoes a series of approximately 15 generations of

divisions and by 20 weeks of gestation form the entire collecting system that is,

the ureter, renal pelvis, calices, papillary ducts and collecting tubules.

At one time it was thought that the ureter forms a solid cord of tissue by

the 6th week of intrauterine life and undergoes canalisation that begins in the

midureteric segment and that extends bidirectionally.(1) The pelviureteric junction

and the ureterovesical junction (UVJ) are the last segments to canalise. More

recently, however, it has been shown that bidirectional recanalisation does not

occur. Recanalisation occurs only at the middle portion of the ureter.(2)

The membranes that develop during canalisation at the pelviureteric

junction or vesicoureteric junction (Chwalla’s membrane) may lead to transient

physiological hydronephrosis or hydroureteronephrosis, respectively. A delay in

the resolution of these membranes can lead to upper urinary tract dilatation.(1,3)

The ability of the kidneys to make urine and to maintain adequate amniotic

fluid volume has a profound influence on the growth and development of the fetus,

particularly after the 18th week of gestation. Under the inductive influence of

ureteric bud, nephron differentiation begins during the seventh week.

By 20 weeks, when the collecting system is completely developed,

approximately one third of neprons are present, nephrogenesis is complete by 36

weeks.

HISTOLOGY

The renal pelvis is composed of three layers; mucosa, muscularis and

adventitia. The inner layer is mucosal and lined by transitional epithelium

supported by a lamina. The epithelium is two to three cells thickness in the renal

pelvis and four to five cells thickness in the ureter. The epithelium sits on a thin

basal lamina which rests in the lamina propria composed of dense fibro connective

tissue with prominent elastic fibres.(95)

The muscular layer is composed of interdigitating fibres of smooth muscle

separated by strands of connective tissue. These muscle fibres are arranged into an

inner longitudinal layer and an outer circular layer.(40)

The adventitia is external to the muscular layer and is composed of

fibroelastic tissue, which is continuous with the capsule of the kidney. The ureter

has rich blood supply with extensive vascular and lymphatic plexus within the

muscularis and lamina propria. Ganglion cells are present within the ureter and

supply motor function to the muscularis. Sensory fibers within the muscularis

penetrate between the cells of epithelium.

PHYSIOLOGY

Urine produced in the glomerulus flows into the pelvis via the calyces. At

physiologic rates of urine production, the calyces and renal pelvic musculature

contracts at a frequency greater than that of the upper ureter.

Morita has demonstrated a relative block of electrical activity at the

pelviureteric junction during normal diuresis.(4) At these flow rates; the pelvis

gradually fills with a concomitant rise in intrapelvic pressure. This continues until

the pelvic pressure exceeds upper ureteric pressure. Urine then flows into the

upper ureter. A ureteral contraction then propels the bolus of urine distally while

the closed pelviureteric junction proximally blocks back flow and prevents

backpressure from the ureteral contraction. Successful conduction of urine from

the renal pelvis requires both anatomic patency and undisturbed transmission of

peristaltic contractions.

PATHOPHYSIOLOGY

Anatomic Response to Urine Flow Impairment

Parenchymal Response

Dilatation of the pelvis and calyces is the first anatomic response to urine

flow impairment. It may lead to significant and long-standing histological damage

of the renal parenchyma and changes in the renal function. This damage is related

to the degree and duration of the urine flow impairment.

The ultimate response to urine flow impairment is renal atrophy due to

programmed cell death called apoptosis.(5) The SGP – 2-gene expression located

in the adventitial layer of the hilar arteries and intrarenal arterioles is responsible

for apoptosis.(6)

Histological Changes

Unilateral ureteral obstruction is characterised by infiltration of renal

cortical interstitial macrophages, an increase in cortical transforming growth factor

– Beta-1 gene expression. Renal cortical TGF- Beta-1 derived from the infiltrating

macrophage, contributes to the formation of the interstitial fibrosis. The release of

prostaglandin E2 and thromboxane A2 may be the responsible for the proliferation

of fibroblast like cells and mononuclear cells.(7) The types I, III, V collagen are

found increased levels in the band of interstitium. Type IV collagen is found in

the tubular basal membrane of the obstructed kidneys.(8)

Histological Changes in the Contralateral Kidney

1. Immunoglobulin G deposits.

2. Increased in size of the glomerular corpuscle.(9)

Timing of Urinary Flow Impairment

The parenchyma response to urine flow impairment is closely related to the

gestational age or the severity of impairment. If it occurs in the early in pregnancy

dysplasia will be formed.(10,11) When urine flow impairment occurs in the later

gestation, it dilates excretory system without affecting the renal parenchyma.(12,13)

Response of the Ipsilateral Excretory System to Urinary Flow Impairment

Ureteral obstruction leads to dilatation and hypertrophy of the pelvis.

Functional Respose to Urinary Flow Impairment

A reduction of the ipsilateral glomerular filtration rate and an increase of

the contralateral glomerular filtration rate is the ultimate response to, significant

and durable unilateral urine flow impairment.

Ipsilateral Vascular Response to Urinary Flow Impairment

The nitric oxide is the prime response for the initial increase in renal blood

flow after acute unilateral obstruction.(14) After the acute phase, the Angiotensin

II, Platelet activating factor and Thromboxane A are responsible for decrease

blood flow and glomerular filtration rate.(15,16) This vascular changes lead to renal

hypoxia, which reflected by an increased level of renal Lactate dehydrogenase in

the obstructed kidney.(17)

Ipsilateral Glomerular and Tubular Response to Urinary Flow Impairment

The affected kidneys produce a larger volume, lose more sodium and have

lower creatinine clearance. The concentrating capacity is also decreased.(18)

Ipsilateral Pelvic Contractility and Pressure Response to Urinary Flow

Impairment

Normal human kidney pelves have uniform peristaltic pressure waves of 1

to 4 mm Hg amplitude and 5 to 10 seconds duration. In patients with mild to

moderate hydronephrosis but well-preserved function, the pressure wave

amplitude and frequency vary only minimally.(19) When function is poor,

however, the amplitude and frequency may drastically change. The baseline

pressure of the normal pelvis also depends on the urine flow rate; the normal range

is 5 to 25 cm of H2O.

Johnston noted that most obstructed kidneys maintain a low intrapelvic

pressure, usually in the normal range.(20) The ability of the extra renal pelvis to

dilate and maintain physiologically normal pressures may help to preserve the

renal parenchyma. This pelvic compliance is effective until a critical volume or

capacity is reached. At this point, smooth muscles, elastin, and connective tissue

were become overstretched. At and above this volume, pressure may increase

sharply.

Koff and Thrall established pelvi -metric curves that correlate pelvic

volume and pelvic pressure and show two phases in cases of urine flow

impairment ; the accommodation phase (pelvic compliance) and the over

distention phase.(21)

Koff (22) describes a pressure dependent flow pattern, seen when intrinsic

pelviureteric junction obstruction is present and characterised by increased flow

with increased intrapelvic pressure. This intrinsic obstruction is caused by a

narrowing or amuscular segment and is associated with the pressure-dependent

restriction to urinary outflow. There is a linear relationship between pressure and

flow such that as pressure in the pelvis increases, so does flow across the

pelviureteric junction.

Koff has also described a volume-dependent flow pattern, seen in extrinsic

pelviureteric junction obstruction. In these patients, flow initially increases with

increased pressure, but this is quickly followed by an abrupt decrease in flow

caused by increasing pelviureteric junction angulation with intrapelvic volume

expansion. As the pelvic volume expands, the pressure also increases, resulting in

increased resistance and even more severe obstruction. Flow remains constant or

declines.

Contralateral Response to Unilateral Urinary Flow Impairment

Due to some biological events after unilateral obstruction, compensatory

hypertrophy occurs in the opposite kidney. (23)

The total number of filtering nephrons however, is decreased in the post-

obstructed kidney indicating a significant increase in the single nephron GFR (SNGFR)

of the remaining nephron units. This is because due to the local production of

prostacyclin and PGE2 and their total effect on the afferent and efferent glomerular

arteriolar vascular resistance. It is obvious that these changes, in turn determine the net

effect on renal clearance or GFR.

ETIOLOGY

The etiologies for the pelviureteric junction obstruction are:

1. Intrinsic abnormalities.

2. Extrinsic abnormalities.

Intrinsic Abnormalities

This is the most common causes of obstruction. They are usually

congenital. The valve like process and polyps may also associate with obstruction

at pelviureteric junction. These prevent urine physically from entering the upper

ureter from renal pelvis. In this abnormality the pelviureteric junction neither was

nor anatomically narrowed and there is no demonstrable extrinsic obstruction.

Histology

Murnaghan noted muscular bundles with abnormal configurations at the

site of obstruction.(24) Decreased musculature was noted. All of these are

consistent with an aperistaltic segment often associated with replacement of the

normal circular musculature with muscle having a primarily longitudinal

orientation. In such instances when the renal pelvis is distended, the ureter will

elongate and the longitudinal bundles will produce narrowing instead of the

normal widening that occurs when circular fibres relax.

Runano-Gil demonstrated that the ureter is the result of the recanalisation

of a tube that is closed during early development. They hypothesized that

pelviureteric junction may be secondarily deformed or incomplete recanalisation.

This theory supported for the hydronephrosis in the fetus that often resolves over

time.(1)

Tainio et al have shown the abnormalities of peptidergic innervation with

dense innervation of neuropeptide Y and vasoactive intestinal peptide and

proposed that these may have a role in intrinsic obstruction (25)

Hanna reported that electron microscopy often reveals disruption of the

intercellular relationship between the muscle cells at the pelviureteric junction.(26)

Extrinsic Abnormalities

Aberrant vessels are seen in about one third of cases.

Stephen coined the term ureterovascular tangle, to describe the condition

where in the proximal ureter is seemingly angulated and obstructed by the aberrant

renal vessels supplying the lower pole of the kidney. (27)A lower hilar segmental

vessel may arise from any point along the course of the main renal artery, aorta

and iliac artery. In 25% of the cases of obstructive hydronephrosis are associated

with this ureterovascular relationship.

This anomaly was uniformly on the left side and the pelvis bulged anteriorly

between the lower and middle hilar segmental vessels. The expanding pelvis

seemed to angulate the adjoining ureter at pelviureteric junction and hook the

ureter over the lower segmental vessels creating a partial obstruction. The

angulated ureter is usually bound by fascial adhesions to the pelvis.

During fetal development the maturing kidney ascends to its position in the

upper part of the retroperitoneum as the renal pelvis changes its orientation from

the anterior to medial. During this stage the kidney acquires its permanent blood

supply. The vessels are arranged in a” ladder” pattern from the aorta to the

involuting mesonephros, and the maturing metanephros tunnels its way toward an

upper retroperitoneal position posterior to these vessels. As the kidney ascends, it

sequentially derives its blood supply from the higher vessel and sheds the lower

one.(28) It is conceivable that abnormal spatial or temporal progression of renal

ascent/ rotation in combination with renal vascular formation may lead to an

unfavorable ureterovascular configuration leading to partial obstruction.

Kinks, bands and adhesions are often intraoperative findings even in the

absence of inflammation or prior infection. These anomalies cause volume

dependent obstruction. As the pelvis distends, the angulation becomes more severe

and the ureter may be folded in such a way that most dependent portion of the

pelvis does not drain. This anomaly then often becomes an apparent high insertion

of the ureter into the pelvis.

CLINICAL PRESENTATION

Pelviureteric junction is the most common site of obstruction in the

urinary collecting system.

Incidence

The incidence of pelviureteric junction obstruction is 1 in 1500 of live

births. The boys are more commonly affected than girls (65%: 35%). (29) In 60%

of cases, the left side is predominate. The bilateral involvement is about 10%.

Today, pelviureteric junction obstruction is manifested in less than 19% of

neonates with mass abdomen.

Before the advent of prenatal ultrasonography most infants presented with

1. Abdominal mass

2. Hematuria

3. Urinary tract infection

4. Gastrointestinal discomfort (30)

Most of the cases now detected during prenatal ultrasonographic screening.

The fetal kidney and collecting system are often detectable by USG scanning as

early as 15 weeks of gestation. In those children with detectable abdominal masses

on physical examination 50% of masses are genitourinary in origin and of these

40% are secondary to hydronephrosis caused by a pelviureteric junction

obstruction. Older children usually present with abdominal discomfort, hematuria

following minimal trauma, nausea or vomiting or urinary tract infection. (31)

Dietl’s crisis, which refers to episodes of pain, vomiting, oliguria with abdominal

lump, is due to intermittent hydronephrosis.(32)

ASSOCIATED ANOMALIES

1. Multicystic dysplastic kidney

2. Duplication of collecting system

3. Imperforate anus

4. Congenital heart disease

5. VATER association

6. Oesophageal atresia

INVESTIGATIONS

1. ULTRASONOGRAPHY

This is the first radiological investigation of choice in a child suspected

pelviureteric junction obstruction. No special preparation is required prior to the

study. In ultrasonogram the following information should be obtained. (Fig

no.4&5)

1. Degree or severity of hydronephrosis

Anteroposterior diameter of pelvis<12mm-mild, 12-20mm-moderate,

>20mm-severe

2. Size of renal pelvis

3. Calyceal dilatation

4. Renal length, ipsilateral and contralateral kidney

5. Cortical thickness

6. Status of the ureter

7. Bladder wall thickness and bladder volume at the time of study

The USG is simple to perform, noninvasive, easily reproducible. It is

important that the child is well hydrated prior to the study. The classical picture

seen in pelviureteric junction obstruction is the Mickey Mouse appearance.

Dhillon et al have measured the anteroposterior diameter of renal pelvis in

the transverse plane in all children with an antenatal diagnosis of hydroneprosis.

This has been unique parameter in the management of antenatally diagnosed

problems.(33)

He also maintain that children with renal pelvis of anteroposterior diameter

<20mm did not come for surgery and progressive increase in the degree of

hydronephrosis may precede functional deterioration on renography after several

years.

Ransley et al showed that none of the children being followed for

asymptomatic hydronephrosis with an AP renal pelvis of <12mm, required a

pyeloplaty.(34)

Koff and co workers used compensatory changes in the opposite normal

kidney as parameter of obstruction in unilateral hydronephrosis.(35)

On USG the longitudinal scan of normal kidney appears as an oval organ

with the echo poor (black) parenchyma and white central echogenic area. The

parenchyma includes both cortex and medulla. Central echogenic area contains

fibro fatty, vessels, lymphatic and most important of all the pelvicalyceal system.

Normally the pelvicalyceal system is collapsed and is not separated. When

there is hydronephrosis, fluid distended pelvicalyceal system is seen on

longitudinal scan as separation of central echogenic area by lucency of fluid. This

separation will increase with increasing hydronephrosis.

On seeing this, next step will be a coronal scan of kidney where the fluid

filled calyces will be seen to join the pelvis. After this, trace the pelvis to continue

as the ureter if obstruction is beyond PUJ. Visualizing the proximal ureter on one

side of the image the ureter is traced down by tilting the probe to find out the level

and cause of obstruction.

Sometimes a vessel crossing the PUJ and causing obstruction can be

visualised on colour Doppler study. A calculus at PUJ will be seen as a bright

echogenic mass in the lumen with acoustic after shadow.

Hydronephrosis is the most common anomaly diagnosed in utero. With

advent of maternal ultrasonography, hydronephrosis is frequently detected is utero

and the diagnostic work up is completed shortly after birth. Before the use of

maternal sonography, infants would present with urosepsis, abdominal masses,

failure to thrive and hematuria. Pelviureteric junction obstruction was the most

common cause of hydronephrosis (22%).

Pelviureteric junction obstruction is suspected when there is dilatation of

the renal pelvis and no visualisation of the ureter. On ultrasonography the dilated

pelvis should also communicate with calices. Lack of communication suggests a

multicystic dysplastic kidney.

It is generally agreed that dilation of the urinary tract as seen in

ultrasonography, IVP or Retrograde pyelography alone is insufficient to diagnose

obstruction of the upper urinary tract. Attempt to use Doppler ultrasound for renal

blood flow studies have been made. For example, the renal blood flow decreases

with obstruction, a parameter called the resistive Index have been studied.

The resistive index is defined as the peak systolic velocity minus the lowest

diastolic velocity divided by the peak systolic velocity.(36) Normal kidneys reliably

demonstrate resistive index < 0.70, and obstructed kidneys show higher values.

Renal resistive index of less than 82% correlate well with intrarenal pressures of

less than 14cm H2O . To expand the utility of this study further, furosemide can be

administered in the course of this study. An increase of 15% in the postfurosemide

resistive index over the prefurosemide resistive index does correlate with the

pressure of obstruction with 88% specificity and 76% sensitivity. Thus far, the use

of resistive index to define the presence of obstruction has not gained widespread

acceptance in paediatric age group.

2. VOIDING CYSTOURETHROGRAPHY

In 9%-14% of patients with pelviureteric junction obstruction have

vesicoureteric reflux. Conversely, 1% of patients found to have PUJ obstruction.

These associations, albeit low, can affect renal function and change management.

Consequently, voiding cystourethrography is the standard of practice for the

clinical evaluation of all infants with prenatal hydronephrosis, regardless of age or

gender.(37,38)

Recently, several investigators have challenged this standard. They

concluded that VCUG should be limited to children with pelviureteric junction

obstruction who also have a dilated ureter on ultrasound.(39)

3. INTRAVENOUS PYELOGRAHY

In general IVP is delayed until after the first month of life because renal

visualisation may be poor when the renal function is poor. The diagnosis of obstruction is

established by IVP when there is dilatation of the renal pelvis, caliectasis and non-

visualization of the ipsilateral ureter. Delayed films are important because with better

pelvic filling they tend to confirm the diagnosis. In established cases, thickness of the

renal parenchyma is reduced and in severe hydronephrosis, a sliver of opacification

known as “Shell Nephrogram” or “Rim sign” is seen.(Fig No.11) In severe

hydronephrosis, a crescentic collection of contrast medium “Crescent Sign” is observed

in the collecting ducts overlying nonopacified calyces. It indicates that some renal

function is present.(40)

The drawbacks of IVP include the necessity of dehydration even in infants,

which makes it a relatively risky procedure. Of course, a risk of radiation exposure

exists, which can be minimised by limiting number of films taken. Problems

associated with contrast media exist, such as nephrotoxicity and anaphylactic

reactions. The newer nonionic contrast agents that are currently available can

reduce these problems.

4. RETROGRADE PYELOGRAPHY

Retrograde pyelography is rarely needed to diagnose pelviureteric

junction obstruction because the diagnosis is ascertained by ultrasonogram and

diuretic renogram. The value of retrograde pyelography lies in the preoperative

evaluation of the ureter to rule out missed ureterovesical obstruction and to

anatomically characterise the pelviureteric junction obstruction.

5. DIURETIC RADIONUCLIDE RENOGRAM

Diuretic renogram is a provocative method of evaluating patients found to

have dilation of the upper urinary tract in which an obstructive process is

suspected. (Fig No.18)

The theoretical basis of this test is two fold: if an obstructive lesion is

present, then (a) renal function, more specifically glomerular function may be

impaired and (b) a dilated upper urinary tract will retain a larger amount of

radionuclide that will not wash out if increased urine flow is generated by the

administration of a diuretic.

Three radio pharmaceuticals are primarily used in diuretic renography and

their characteristics are linked to their biological activity. Technetium 99m

Diethylenetriaminepentaacetic acid (99mTc-DTPA) and technetium 99m-

Mercaptoacetyltriglycine (MAG3) are preferentially concentrated by the kidney

and filtered by the glomerulus.(41)

DTPA is neither secreted nor resorbed by the renal tubules, where as

MAG3 is secreted by the tubules. Because each is nearly completely excreted,

they can be used to estimate differential function and urinary drainage. The third

agent, Tc99 dimercaptosuccinic acid (99m TcDMSA), is tightly bound to renal

tubular cell and is there fore, useful for the detection of differential renal function

and clinically significant cortical lesions such as renal scars.

In general, neonates and young infants are placed supine for the study.

Ideally, the child should be well hydrated because relative dehydration prolongs

parenchymal transit and delays urinary excretion.

MAG- 3 or DTPA is injected intravenously as a bolus. The paediatric dose

of MAG-3 is 50mcu/ kg. (43) Subsequently, 4- second posterior images are

recorded with a high-resolution collimater. One minute after injection, images of

the kidneys are obtained each minute.

Normally, the renal parenchyma is well visualised during the first minute;

by 2 (or) 3 minutes, activity seen in collecting system and by 6 to 9 minutes the

bladder is visualised.

The differential renal function of each kidney is calculated between 60 and

180 seconds after injection of the tracer, which represents parenchymal transit and

refects glomerular function. On computer images, region of interest (ROI) are

selected from each kidney and background activity is substracted.

The activity within each kidney is expressed as a percentage of the total

renal counts, and this differential activity is used to compute differential renal

function.

To determine whether significant obstruction is present, furosemide is

administered intravenously. There are 3 variations in the furosemide

administrations.

1. F +20 - furosemide is injected 20 minutes after the injection of tracer.

2. F – 15 - furosemide is injected 15 minutes prior to the tracer.

3. F – 0 - furosimide is injected at the beginning of the study

The most commonly used protocol for diuretic renogram is that in which

the furosemide is administered 20 minutes after tracer injection the so-called F+20

protocol. The dose of furosemide in paediatric is 1mg/kg in infants, 0.5mg/kg in

children aged 1-16 years.

Furosemide is a potent loop diuretic that induces acutely increased urine

flow, reaching maximal effect 15 to 18 minutes after intravenous administration.

The furosemide is injected slowly over 3 minutes. Images are acquired for at least

20 minutes following injection.

At completion of the study, a static upright post void image is obtained,

especially if the study has been performed supine and persistent tracer pooling

seen within the renal pelvis.

Image acquisition must be carried out for at least 20 minutes following

furosemide injection to avoid missing flow- dependent intermittent obstruction the

so called “ beer drinker’s kidney”.

The renogram curve in this setting may appear to fall normally. In

response to furosemide early on, but, as urine flow rates reach maximum, the

curve again deflects upward in an obstructive pattern. This produces the “delayed

double peak” pattern – Homsy’s sign of intermittent obstruction.(42) Premature

termination of the test may result in missing this second upward curve deflection.

In these patients, a follow up F – 15 renogram is helpful in confirming the true

obstruction. (43). F - 15 renography can be used in patients in whom the

diagnosis of significant obstruction is equivocal by F + 20 protocol.

A combined approach has also been recommended in patients in whom

obstruction is clinically suspected but in whom most of the tracer activity has

drained from the collecting system at the end of the F + 20 renogram. In such

instances, a repeat injection of the same dose of tracer is immediately made and

another 20 minutes study acquired.

The additional information, which can be obtained, includes clearance half

time of the tracer from the pelvicalyceal system, which is used by some workers as

a parameter to diagnose obstruction.

The renogram curve

Diuresis renography was pioneered by PH ‘O’ Reilly (1978) and associates

as a means of distinguishing between obstructive and non obstructive

dilatation.(44)

The characteristics of the uptake and drainage curves fall into four patterns;

Type 1: Normal uptake with prompt washout.

Type 2: Rising uptake curve, no response to diuretic.

Type 3a: An initially rising curve, which falls rapidly in

response to injection of lasix.

Type 3b: An initially rising curve, which neither falls promptly

Following the injection of lasix nor continues to rise.

This renogram pattern was defined by O’Reilly and

Colleagues as equivocal and much of their subsequent

work has been aimed at eliminating the type 3b curve

by modifying the technique and timing of lasix administration

The renogram curve

A. Normal curve shows the three renogram phase.

B. Abnormal curves. These curves are typical for obstructive or nonobstructive

dilatation with accumulation of tracer in the collecting system. Impaired renal

function with cortical retention of tracer can produce a similar appearance,

although the paek parenchymal activity will be lower than normal.(bottom curve)

C. In non obstructed collecting system, there is prompt washout of pelvicaliceal

activity after intravenous furosemide.

D. In case of obstruction or impaired renal function,poor or no washout is seen.

The renogram curve is a time activity curve describing the transit of tracer

through the kidney. The curve is obtained by placing a computer assisted region of

interest over the whole kidney or cortex obtaining the counts in the ROI for each

period of data acquisition and plotting these counts as a function of time.

The renogram curve is often divided into the period of tracer appearance;

tracer extraction and tracer elemination denoted as phase 1, 2 and 3 respectively.

Tracer appearance describes the period of blood flow beneath the detector; tracer

extraction is proportional to renal plasma flow or glomerular filtration rate

according to used tracer. The curve peaks when tracer exits from the kidney at the

same rate it is entering the kidney. Normally the intrarenal transit of tracer is less

than 5 minutes.

The excreting part of the curve is measure of drainage from the pelvis.

Various quantitative parameters are available but the simple one is measuring the

half time (T1/2) of excretion. It is generally recognized that a T1/2 < 10 minutes is

considered normal, a T1/2 between 10-20 minutes is considered indeterminate and

a T1/2 of >20 minutes is likely caused by an obstruction.

However, interpretation of the T1/2 should not be attempted without

evaluation of the dynamic images, renal curves and other quantitative factors. The

technique was also compared with other modalities like perfusion studies

described by Whitaker where 85% correlation was obtained. When compared with

morphological studies (Goslings and Dixon 1978) the correlation rate was 88%.

Factors affecting diuretic renography in the neonate are

Renal maturity Volume of urine in the bladder

Renal functioning Outlined regions of interest

Hydration status Patient position

Type and dose of tracer Patient movement

Dose of diuretic Capacity of upper tract

Timing of diuretic administration Severity of obstruction

Vesicoureteric reflux Site of obstruction

Method of data interpretation

It has become the procedure of choice in the evaluation of suspected

obstruction because it is relatively noninvasive, providing information about

differential function and reliably diagnosing functional obstruction.

Glomerular filtration can be measured by determining the late of its

removal from the blood. The 99Tc emits a 140Kev gamma ray with no harmful

beta emission. The estimated absorbed dose by the kidney is 0.04 mrad and total

body absorption is 16 mrad, which is lesser than intravenous pyelogram..

To reduce inter institutional difference the society for fetal urology and

society of nuclear medicine council proposed a uniform methodology for

performing the diuretic renogram called well - tempered renogram.(45)

To reduce the likelihood of immature renal function interfering with

interpretation, infants should be older than 2 weeks. Oral hydration is begun 2

hours before study. Intravenous hydration of 15ml /kg over 30minutes is started as

a bolus 15 minutes before injection of the isotope followed by maintenance fluid

at a rate of 200ml /kg/24hours.The bladder is catheterised throughout the study.

Diuretic renogram is well tolerated, easily repeatable and appropriate for

paediatric patients. It should be done after 4-6 weeks of life.(46)

6. CT SCAN

CT scan has been used to diagnose pelviureteric junction obstruction in

children, especially in association with the abdominal trauma. CT scan, like IVP

studies, shows the dilatation of the kidney and collecting system well, and it may

be used to estimate the differential renal function by measuring the cortical

thickness.(Fig No.9&13) CT angiogram, Helical CT scans are used to evaluate the

presence of lower pole vessels preoperatively.

7. MAGNETIC RESONANCE IMAGING

New developments in MRI technology have made it possible to image

kidneys while assessing intracellular metabolic parameters independent of blood

flow and tubular function. Relative high cost and the noise during the procedure

limit the routine use of MRI for evaluating urinary obstruction in children.(Fig

No.15)

8. ENDOURETERAL SONOGRAPHY

Bagley and co workers have used endoureteral sonography in the

evaluation of the obstructed pelviureteric junction.(47) Catheter based ultrasound

probes with single crystal transducers (12.5 Mhz/ 20Mhz) are available in size

ranging from 3.5 F to 6.2 F. The transducer is densely radio opaque and can be

seen fluoroscopically. The device can be used in conjunction with standard

endoscopic equipment. It can provide accurate information of pelviureteric

junction anatomy. It can visualise vessels adjacent to the PUJ/ ureter; define the

presence of high ureteral insertion including the direction, length and thickness of

the septum.

With increasing popularity of endoscopic treatment techniques, this

modality may ultimately provide the key to the selection of the optimal therapy for

the obstructed pelviureteric junction.

The obstructed PUJ has three distinct patterns seen sonographically. The

area of the PUJ can be quite narrow, with a stenotic segment ranging from 1 or 2

to several millimeters.

This can be the only finding or may be associated with the other two

patterns of crossing vessels or high insertion, as the presence of one pattern does

not exclude the others.

9. PRESSURE PERFUSION STUDY

Whitaker’s test was considered the gold standard for the evaluation of

upper urinary tract dilatation.(48) It provides urodynamic evidence of a mechanical

obstruction of the upper urinary tract at a given flow rate. With the advent of the

diuretic renogram and some of the newer radiopharmaceutical agents, the

Whitaker test is not often utilised clinically.

Whitaker test is performed with the patient placed on a fluoroscopy

table in the prone position. Before the patient is positioned prone, a bladder

catheter is placed and connected to a pressure transducer for continuous

monitoring of intravesical pressures with changes in renal pressure. A renal

cannula (18-gauge) is then inserted and connected to a pressure transducer. A

combination of saline solution and contrast material is administered by way of the

renal cannula at a rate of 10 ml/min. Bladder pressure is monitored throughout the

procedure, and its relationship to changes in renal pressure can be significant.

Contrast material is given along with the saline solution, making fluoroscopic

monitoring of the anatomic site of the obstruction possible .Whitaker’s test

Results are separated into three categories

1. Pressure less than 15 cm H 2 O = non obstructed

2. Pressures of 15 to 22 cm H 2 O = equivocal

3. Pressures greater than 22 cm H 2 O = obstructed

Because the diuretic renogram is noninvasive, easily reproducible and

provides quantitative evaluation of the split and total renal function with minimal

radiation exposure, it is clinically utilised more today than the Whitaker test.

However, when there is extreme upper tract dilatation or poor renal function or

both, precluding an adequate diuretic response the Whitaker’s test may still have

clinical utility.

10. URINARY MARKERS PELVIURETERIC JUNCTION OBSTRUCTION

1. Urinary Beta 2 Microglobulin

Disruption of proximal tubular integrity leads to increased urinary

concentrations of beta-2-microglobulin (B2M), which normally reabsorbed from

the tubular lumen via phagocytosis and lysosomal digestion.(49)

An increase in urinary concentrations of B2M may indicate tubular

dysfunction as a result of the obstructive insult. Functionally significant

obstruction and recovery from obstruction may be determined by following the

urinary concentration of B2M.

The potential for B2M to be a marker for significant obstruction is quite

appealing; however, the determination of its levels in obstructed kidneys is not routine,

and many different insults other than pelviureteric junction obstruction can lead to

increased levels of B2M in the urine. In addition, the immaturity of the nephron and the

high fractional excretion of water in neonates contribute to elevated B2M levels in the

absence of any identifiable renal stress.

2. N-Acetyl-β- Glucosaminidase

N-acetyl-β-glucosaminidase (NAG) is a tubular lysosomal enzyme present

in the urine of children who have various renal diseases. This is currently

experimental.(50)

In rats with experimental partial ureteral obstruction, the urinary

concentration of NAG increases in the first 2 weeks of obstruction and decreases

with the relief of obstruction.

Urinary biochemical markers of renal damage sometimes may aid the

diagnosis of clinically significant urinary obstruction.

The assessment of urine for growth factors (eg, epidermal growth factor

[EGF], platelet-derived growth factor [PDGF], TGFb, cytokines, p53, p21) and

vasoactive substances may be an important adjunct in evaluating obstructive

uropathy in the future.(51)

TREATMENT

Goals

The goals of the treatment of pelviureteric junction obstruction are to

improve urine flow to prevent further parenchymal damage and to alleviate

symptoms when they exist. There are four therapeutic approaches are available for

the treatment of PUJ obstruction

1. Conservative management

2. Temporary diversion of urine (percutaneous nephrostomy)

3. Surgery or endoscopic treatment

4. Fetal surgery or urinary diversion

Conservative Management

Conservative management of pelviureteric junction obstruction is justified

in most of the cases during the first year of life. Three conditions are required to

treat a unilateral pelviureteric junction obstruction conservatively.

1. The asymptomatic child

2. The pelvic dilatation should be stable or decrease on repeated

ultrasonogram

3. The relative function on repeated isotopic studies should be stable or

increase.

Temporary Diversion

Temporary diversion of the urine is indicated in infants with severe

unilateral pelvic dilatation and poor relative function. The percutaneous

nephrostomy is recommonded for 3 to 4 weeks, followed by reassessment of

relative function. At this point, if the function is improved, pyeloplasty will be

considered or the function remains poor, nephrectomy should be discussed. In case

of bilateral dilated pelvis, unilateral nephrostomy usually improves drainage from

both kidneys.

Surgical or Endoscopic Treatment

Indications

1. A symptomatic pelviureteric junction obstruction

2. Declining function of the dilated kidney

3. Increasing pelvic dilatation

4. Bilateral, moderate to severe dilatation of the pelvis

Surgical Treatment

PYELOPLASTY

Historical review

The first PUJ repair was performed by Trendelenburg in 1886. The first

successful pyeloplasty is credited to Kuster in 1891. In 1894 Fenzer applied the

Heineke- Mickulicz principle to reconstruct the pelviureteric junction. The

dismembered procedure of Kuster was modified by Nesbit in 1949 and further

modified by Anderson and Hynes by spatulating the ureter and excising the

redundant pelvis.

The flap procedure was introduced by Schwyzer in 1823. This is further

modified by Foley who described the Y-V plasty in 1937. This technique was best

applicable to cases of high insertion and unsuitable for pelviureteric junctions,

which were dependent.

Four criteria for success in the repair of a pelviureteric junction obstruction

were defined by Foley in 1937 as follows (52)

1. Formation of a funnel

2. Dependent drainage

3. Water tight anastomosis

4. Tension free anastomosis

The following procedures are available to treat pelviureteric junction obstruction

1. Dismembered Procedures

- Open Anderson Hynes pyeloplasty

- Laparoscopic pyeloplasty

2. Flap Procedures

- Foley Y plasty

- Spiral flap

- Scardino Prince Vertical flap pyeloureteroplasty

3. Ureterocalycostomy

4. Nephrectomy

Dismembered Procedures

Dismembered procedures involve excision of pelviureteric junction with an

anastomosis of the upper ureter to the dependent renal pelvis. Success rate of this

procedure ranges between 90-95%, with failure often due to fibrosis secondary to

urinary extravasation or to poor tissue handling.

The Anderson-Hynes pyeloplasty has become the most commonly

employed "open" surgical procedure for the repair of pelviureteric

obstruction.(53)The principal reasons for the universal acceptance of the

dismembered pyeloplasty are

1. Broad applicability, including preservation of anomalous vessels

2. Excision of the pathologic pelviureteric junction and appropriate

repositioning

3. Successful reduction pyeloplasty.

This operation is generally easy to perform and can be accomplished by a

number of surgical approaches including

1. Anterolateral extraperitoneal approach

2. Posterior lumbotomy approach

3. Flank approach

Anterolateral extraperitoneal approach

Anderson-Hynes dismembered pyeloplasty, as performed through an

anterolateralaproach, is as follows (Fig No.20)

1. The anterolateral incision is a muscle-splitting incision that is made with

the patient supine and a roll placed transversely beneath the patient to

elevate the flank.

2. Each muscle layer encountered is split in the direction of the muscle fibers

until Gerota's fascia is identified by sweeping the peritoneum medially. The

fascia is then incised posteriorly over the lateral aspect of the kidney.

3. The renal pelvis is identified by medial retraction of the peritoneum and

lateral traction of the kidney.

4. Anterior exposure is usually better when a dismembered pyeloplasty is

being performed. Once the ureter and pelviureteric junction are identified,

a traction suture is displaced anteriorly through the proximal ureter to

minimise subsequent handling.

5. The area of pelviureteric junction is dissected free to allow for a clear

area in which to perform the anastomosis. Traction sutures may be placed

in the renal pelvis superiorly, medially, laterally, and inferiorly to the

pelviureteric junction. Once adequate ureteral length is confirmed and the

pathology of pelviureteric junction identified, the ureter can be transected at

this level.

6. The ureter is spatulated on the side opposite to the traction suture using

Potts tenotomy scissors. The distance over which the ureter is opened is

variable, until healthy ureter is encountered, which springs open when

forceps are placed into it.

7. A portion of pelvis is excised. It is better to leave too much renal pelvis

than too little, especially when resecting along the medial aspect of the

renal pelvis. The ureter and renal pelvis are aligned to ensure that the

anastomosis can be accomplished without tension. If a nephrostomy tube is

to be used, it is placed at this time. An inferior calyx is chosen, preferably

where the overlying parenchyma is not too thick.

8. The anastomosis is started by placing the first suture at the apex of the "V"

in the ureter and into the tip of the inferior pelvic flap. As the suture is tied

down, the ureter and renal pelvis are brought together to minimise tension

on the repair. A small feeding tube is placed into the ureter; it can be used

to stabilize the ureter during the anastomosis. A "no-touch" technique is

employed with the ureter to minimise trauma and edema to the ureteral

tissue. The area of the initial anastomosis is critical to ensuring a watertight

closure.

9. Before the repair is completed, the renal pelvis is irrigated to remove any

blood clots or debris that could obstruct the pelviureteric junction. A drain

is placed adjacent to the repair and brought out through a separate stab

wound.

10. The kidney is returned to its native position, and perinephric fat if available,

is placed over the anastomosis. Wound closed in layers.

11. The transanastomic stent removed usually after 48hours. The drain

removed on 4thday.

Anderson-Hynes pyeloplasty

In the following situations, postoperative temporary nephrostomy is advisable.

1. Inflamed renal pelvis (prior nephrostomy, presence of calculus, infection)

2. Thin or hypoplastic ureter.

3. Operative difficulties.

4. Poor renal function.

5. Solitary kidney or bilateral disease.

6. Transabdominal pyeloplasty.

7. Long segment stricture or distal ipsilateral pathology (VU reflux and when

PU junction edema anticipated)

Posterior lumbotomy approach

Posterior lumbotomy approach is more commonly used in infants, small

children and lean older preadolescent with normally located operative kidney.(54)

The use of muscle splitting rather than cutting makes it almost a minimal invasive

procedure. The location of the incision is posterior and in the crease line has a

cosmetic advantage.

Procedure

The child is placed in a prone position with a roll under the chest and

midthigh regions. A transverse skin incision is made just under and parallel to

the 12th rib, with one third of the incision over the paraspinal muscle and two third

lateral to the skin. Scarpa's fascia is sharply incised and a vertical incision is made

through the lumbodorsal fascia (posterior lamella).

The lateral edge of the lumbodorsal fascia is elevated, and the sacrospinalis

muscle is medially retracted. An incision is made through the middle and anterior

lamella of the lumbodorsal fascia, taking care not to injure the ileohypogastric

nerve. The quadratus lumborum muscle is retracted, exposing Gerota's fascia

beneath the paranephric fat, and then this fascia is opened.

The renal pelvis is identified, and several holding stitches are placed in the

pelvis. The ureter is identified, a holding stitch is placed in the ureter, and the

surgeon proceeds with the dismembered pyeloplasty as usual manner.

After pyeloplasty, a single muscle fascia layer bringing the lumbodorsal

fascia back together again does closure.

The bilateral procedure can be done successfully under same anesthesia

without position changes or redraping. Pain is minimal, morbidity reduced and

mobilisation is almost immediately.

Flank Approach

The patient is placed over the kidney rest in a flank position; the kidney rest

is elevated and the operating room table is flexed. The skin incision is made off

the tip of the 12th rib, or, if necessary, a supracostal 12th rib incision is made.

The external oblique and latissimus dorsi muscles are divided. Next the

internal oblique and serratus posterior inferior muscle are divided. The

transversalis muscle is often thin and can be divided with digital dissection. The

peritoneum is identified and retracted medially.

Gerota's fascia is then encountered and opened longitudinally to gain exposure to

the perinephric space. After identification of the renal pelvis and the ureter, a

dismembered pyeloplasty can be performed as described earlier.

Flap Procedures

Culp and DeWeerd's spiral flap (55) can be used for patients with a

dependent pelviureteric junction and a relatively long area of proximal ureteral

obstruction.

The spiral flap is outlined with its base situated obliquely on the dependent

aspect of the pelvis. The base of the flap is positioned anatomically lateral to the

pelviureretic junction and should lie between the ureteral insertion and the renal

parenchyma. The flap is then spiraled posteriorly to anteriorly or vice versa. The

medial line of incision through the flap is carried down completely through the

obstructed proximal ureteral segment into normal caliber ureter.

The length of the flap is determined by the site of the apex, which in turn

should be a function of the length of proximal ureter to be traversed. In order to

insure vascular integrity of the flap, however, the ratio of flap length to width

should not exceed3-1. The flap is developed with fine scissors and the apex then

rotated down to the inferior most aspect of the ureterotomy. The anastamosis is

completed over an internal stent, using fine absorbable sutures.

Scardino and Prince described a vertical flap that can be used in the

situation of a dependent pelviureteric junction with a large, square-shaped

extrarenal pelvis. (56)

In contrast to a spiral flap, however, the base of the vertical flap is situated

horizontally rather than obliquely between the pelviureteric junction and renal

parenchyma. The flap itself is formed by a convergence of two straight lines from

the base vertically to the apex on either the anterior or posterior aspects of the

renal pelvis.

As for the spiral flap, the height of the apex determines the length of flap

obtained which again is a function of the length of proximal ureter to be bridged.

Medially, the ureterotomy is carried through the proximal ureter completely

through the strictured area, continuing a few millimeters into normal caliber

ureter. The apex of the flap is rotated down and joined to the inferior most aspect

of the ureterotomy. The flap is closed over an internal stent by approximating the

edges with fine absorbable suture.

Foley -Y- Plasty

This is useful technique when the obstructed segment is more than 2cm in

length a flap of renal pelvis is rotated inferiorly to restore ureteral caliber.(52)

In this procedure, the limbs of the Y are widely separated one on the

anterior and one on the posterior aspect of the renal pelvis. The laterally placed

portion of the pelvis then drops inferiorly. The lateral border of the ureter is

opened longitudinally through the pelviureteric junction to at least 1cm below

the obstruction where the ureteral caliber is normal. The midportion of the pelvic

flap is sutured to the inferior margin of the ureterotomy converting the incision to

a V.

Ureterocalicostomy

This procedure generally reserved for situations in which dependent renal

drainage cannot be established by conventional techniques. It is primarily used for

horseshoe kidney, fusion anomalies and in the repair of secondary pelviureteric

junction obstruction. The inferior calyx is more dependent than pelvis in the

Horseshoe kidney and it may be a more physiologic procedure than pyeloplasty.

According to Hawthorne et al cardinal features of a successful

ureterocalycostomy should include amputation of enough lower pole cortex to free

the ureter from entrapment by contracting fibrosis of the renal cortex. (57) There

should be no tension on the anastomosis and there should be no renal parenchyma

below the level of the repair.

Nephrectomy

Because the recovery potential of the kidney is greater in children,

extreme conservation is justified. Salvage pyeloplasty should be considered as

renal function shown in renal scintigraphy can recover.(58) During surgery, the

renal cortex should be assessed. The severe cystic dysplasia is an indication for

nephrectomy, otherwise every effort be made to salvage the kidney.

In the bilateral anomalies, unilateral pyeloplasty on the most severely

dilated pelvis is usually recommended. Unilateral pyeloplasty often improves the

dilatation of the contralateral side as well. If the dilatation of the contralateral side

does not improve, pyeloplasty should then be considered. If nephrectomy is

considered on one side, the pyeloplasty should precede this.

ENDOSCOPIC MANAGEMENT OF PELVIURETERIC OBSTRUCTION

Historical review

The idea of endopyelotomy was conceived in 1909 by Albarran in France

describing the technique of incising the narrowed pelviureteric junction as

URETEROTOME EXTERNE in which a stent was left in.(59) The incised ureter

heals over time, which is something akin to internal urethrotomy in stricture

urethra. Davis 1943 popularised this technique as intubated ureterostomy in which

few loose stitches were placed.(60)

Wickham and Miller first described a percutaneous approach to incising

the pelviureteric junction termed pyelolysis. Subsequently Arthur smith

popularized this technique renaming it endopyelotomy.(61,62)

With the advent of endourological techniques the following minimal

invasive procedures are being applied to pelviureteric junction obstruction

1. Antegrade nephroscopic endopyelotomy

2. Retrograde ureteroscopic endopyelotomy

3. Balloon dilatation with cutting device

1. Antegrade Endopyelotomy

Open surgery has been the optimal therapy for pelviureteric junction

obstruction, and the dismembered pyeloplasty remains the gold standard.

Percutaneous techniques were introduced in the early 80’s for the management of

nephrolithiasis. Subsequently in 1983 reports began to appear in the literature for

endoscopic treatment of pelviureteric obstruction. Success rates vary from 50% to

95% and remain inferior to that of open pyeloplasty.(63) Success appears to be

dependent on correct patient selection rather than the type of operation.

Patient Factors

1. Age

Neonates and infants may not be suitable candidates for endourologic

management. In addition to technical difficulties, the radiation exposure may be

extensive. Children in the adolescent age group are more suitable as the anatomy

and caliber of the ureter is similar to the adult urinary tract.

2. Hydronephrosis

Van Cangh (64) in his series of adult patients found the success rate of

endopyelotomy fall from 95% to 77% in the presence of massive hydronephrosis.

3. Crossing Vessels

Van Cangh reported the success rate of endopyelotomy was only 42% in

the presence of crossing vessels and dropped to 39% in the presence of both

crossing vessels and massive hydronephrosis.

4. Primary versus Secondary PUJ Obstruction

A review of most recent series including that of Van Cangh and co

workers, it appears that secondary UPJ obstruction responds better to antegrade

pyelotomy.

In older child or adolescent patient with primary pelviureteric junction

obstruction and favorable factors (e.g. good ipsilateral renal function and only

mild to moderate hydronephrosis), endopyelotomy is the preferred procedure.

Endopyelotomy is the initial therapy for secondary PUJ obstruction in all-pediatric

patients

Endopyelotomy

The pelviureteric junction can be incised with various devices.

1. Cold knife.

2. Electrocautery.

3. Laser – Nd YAG / KTP / Holmium

The UPJ is assessed to determine whether it is wide enough to

accommodate subsequent instrumentation. If it is stenotic it can be dilated using a

balloon.

The Incision

This is made on the lateral side and extends down the ureter for a

centimeter beyond the area of narrowing. Proximally it extends 1-2 cm up the

pelvis and should go through all layers till the retroperitoneal fat is visualised.

Either a graduated or a standard stent is passed down the guide wire into the

bladder. A nephrostomy tube is placed in the pelvis and remains for 48 hours. A

nephrostogram is usually performed prior to removal and the stent is left

indwelling for 3-6 weeks.

2. Retrograde Ureteroscopic Endopyelotomy

A 5 or 6 F indewelling ureteral stent is placed 1 to 2 weeks prior to

planned endopyelotomy. This stent drains the obstructed kidney and stabilises

renal function. This maneuver facilitates passage of the ureteroresectoscope and

allow for the preoperative evaluation of stent intolerance. Under flouroscpic

control retrograde pyelography indicates the level and extension of pelviureteric

narrowing using ureteroscope of appropriate size according to size of ureter. The

PUJ narrowing is incised at the lateral position (8to9 0’clock on the right, 3to4 0’

clock on the left) and stent is left indewelling. Incision at the lateral position

minimises the risk of injury to any possible crossing vessels. The success rate is

75% in primary and 82% for secondary PUJ narrowing.(65)

3. Retrograde Balloon Cautery Incision of the Pelviureteric

Junction (Acucise Device)

A special balloon catheter with an inbuilt cutting device is used in this

technique. Once the waist disappears (after 10 minutes of balloon dilatation)

cutting current is activated for 3 seconds to get the desired endopyelotomy.

Overall success in this method is around 78% and hospital stay is around 1 day

only. In this method the retrograde balloon is used to define the area of stenosis

and to carry the cutting wire into the area to be incised. It is designed to accept a

maximum of 2.5ml of fluid. The electrically active surface on the cutting wire is

2.8cm in length and 150µm in diameter.

Balloon inflation with contrast indicates the narrowed PUJ segment by

presenting as a waist. Then the cutting device which is positioned across the PUJ

in such a manner that it points posterolaterally, is activated by cutting current for 3

seconds, so as to cut the stenosed PUJ precisely but surely in one plane only

avoiding the vessels.

After completion of the incision, retrograde pyelography is performed

through the retrograde balloon catheter to confirm extravasation at the incision

site. The ureteral stent should be kept for a period of 6 weeks postoperatively.

Patients return 4 weeks following stent removal for postoperative intravenous

pyelography to confirm efficacy of the endopyelotomy.

Postoperative bleeding, necrosis of ureter, urinoma, hematoma and urinary

tract infections are the frequent complications of this procedure.

Advantages of this technique include

1. Short operating time.

2. Ability to perform it as a day care procedure.

3. No special instrumentation required. The balloon can be passed through a

standard cystoscope.

4. The device can also be used through an antegrade percutaneous

nephrostomy tract.

4. Balloon Dilatation / Endoburst

The advantages of this procedure are similar to that of the acucise device;

however one cannot incise the pelviureteric junction. Cystoscopy is performed and

a 0.035 – inch guide wire passed up the affected ureter and coiled in the renal

pelvis. An appropriately sized dilating balloon catheter (12F to 24F) is passed over

the guide wire and positioned across the PUJ fluoroscopically. The balloon is

inflated for 3 minutes and the PUJ ruptured. Extravasations of contrast confirms

the rupture and a 6 to 8 F stent is positioned over the guide wire on removal of the

balloon catheter.

Tan reported on the early results of balloon dilatation for primary

pelviureteric junction obstruction in 10 children. The age range was 3 months to 9

years and at 22 months follow up had a success rate of 70%.(66)

Pros and cons in endopyelotomy

The concern in high insertion pelviureteric junction endopyelotomy may

not yield good results. It is not a suitable method in vessels crossing pelviureteric

junction where there can be torrential bleeding. Lingman et al 1993 reported

antegrade approach with 100% success when followed for one year. Figenshau’s

4 years follow up shown 100% success. (67)

In children with secondary pelviureteric junction obstruction, the results

are more encouraging. It approaches nearly 100% as against available (70 to 95%

success) results in primary pelviureteric junction obstruction.

Endopyelotomy has proven to be preferred method in failed pyeloplasty

where the success rate is nearly 95 to 100%.

For primary pelviureteric junction obstruction with pelvic size less than

60ml volume, if vessel crossing pelviureteric junction is ruled out by spiral CT

scan or endoluminal US scan, definitely endopyelotomy can be attempted.

LAPAROSCOPIC PYELOPLASTY

The latest technique is laparoscopic pyeloplasty where dismembered

pyeloplasty is done as in open with the advantage of minimal invasive nature.

Laparoscopic pyeloplasty was first reported by Schuesler.(68)

Approach of laparoscopic pyeloplasty

The exposure of pelviureteric junction can be done either by

1. Transperitoneal approach

2. Retroperitoneal approach

By transperitoneal approach pelviureteric junction is exposed by mobilising

the colon. But problems in transperitoneal approach are

1. Transgressing the peritoneal cavity carries the potential risk of

adhesion.

2. Anastomotic leak can cause intraperitoneal spillage, paralytic ileus and

septicemia.

Peters and associates reported the first laparoscopic dismembered

pyeloplasty in a child, using a transperitoneal approach with four ports and

interrupted sutures tied intracorporeally.(69)

Technique

The principle of the dismembered pyeloplasty is followed in general. The

preoperative enema to empty the colon, nasogastric tube to decompress the

stomach and Foleys catheter to keep the bladder empty are helpful to improve the

available space in the peritoneal cavity in children.

Following induction with general anaesthesia, cystoscopy and retrograde

pyelography are performed to confirm the diagnosis and a long indwelling ureteral

stent is passed

The patient is placed in a 45-degree lateral decubitus position and secured

to the operating table. Insufflation is performed through a veress needle and three

laparoscopic ports are passed into the peritoneal cavity.

The ipsilateral colon is reflected and the proximal ureter and renal pelvis

are identified and fully mobilised. If a crossing vessel is present, fibrotic bands

between the vessels and collecting system should be divided. The renal pelvis is

transected circumferentially above the PUJ and the proximally spatulated through

the level of the PUJ laterally. Care should be taken not to cut the ureteral stent.

If the crossing vessels are present, the ureter and renal pelvis are

transposed to the opposite side of the vessels prior to completion of the

anastomosis. All intracorporeal suturing is performed using the endostich device

and 4’0’ poyglycolic acid suture. A corner stitch is placed through the most

dependent portion of the renal pelvis and through the corresponding corner of the

spatulated ureter. The posterior anastomosis is then performed using multiple

interrupted sutures followed by the anterior anastomosis.

As in true open pyeloplasty, the goal of surgical repair is to create a

dependent, tension free, water tight anastomosis.

Following completion of anastomosis, a 5mm closed suction drain is placed

through a posterior stab incision into the perinephric space adjacent to the PUJ.

Hemostasis is confirmed, the CO2 is evacuated and port sites are closed.

RETROPERITONEAL PYELOPLASTY

With the patient in the lateral position tip of 12th rib is identified and iliac

crest is outlined. Between these two points an incision is made in the middle. It is

deepened by a blunt dissection with a hemostat and index finger to reach the

peritoneum.

Then, peritoneum is pushed away from abdominal wall either with index

finger or Hegar dilator (size 12 or 14). The inflating balloon is inserted through

this and kept between the peritoneum and muscles. It is inflated with co2 or saline

is put to create a space beneath the peritoneum. Balloon is kept for few minutes

and is deflated.

A trocar either a Hassons type or a trocar with self-retained balloon at the

tip, is chosen to prevent leak from the trocar site. After verifying the correct entry

of retroperitoneal space by telescopic examination, two additional ports are made

one near the costal margin and another near ileac crest. By blunt and sharp

dissection pelviureteric junction is exposed and pyeloplasty is done in the usual

way.(100)

In retroperitoneal approach, the main advantage is avoiding entry of

peritoneal cavity and thereby urinary leakage related problems can be avoided.

The major disadvantage is very much limited retroperitoneal space in

children and hence there is difficulty in suturing and knotting. In addition creating

retroperitoneal space is difficult when compared to transperitoneal approach.

Complications of laparoscopic pyeloplasty.

The complications are similar to open pyeloplasty like anastomotic leak,

septicemia in the immediate postoperative period and anastomotic stenosis later.

With experience and improvements in instrumentation and innovative

suturing techniques laparoscopic approach will become the procedure of choice.

ROBOTIC PYELOPLASTY

Robotics is another exciting and evolving area for minimally invasive

surgery in children. This new technique provides excellent three dimensional

visualisation, unprecedented control of endocorporeal instruments, and an

ergonomic surgeon’s position. Robots may advance minimally invasive surgery by

allowing paediatric urologist with limited laparoscopic experience, to rapidly

master the endocorporeal skills necessary to treat pelviureteric junction

obstruction.(70,71)

FETAL INTERVENTION

Currently it is accepted that antenatal therapeutic intervention is beneficial

only in bilateral, moderate or severe hydronephrosis, hydronephrosis detected

around 24to28 weeks, fetal renal function is adequate, and there are no associated

serious anomalies. The male child with posterior urethral valves with good renal

function is the usual candidate. Mild uropathy which worsens on serial scans as

shown by increasing dilatation or decrease of renal function is also an indication

for antenatal decompression. The percutaneous vesicoamniotic shunting (in

bladder outlet obstruction),open vesicostomy and open pyelostomy can be used

for antenatal decompression.

PYELOPLASTY FOR SPECIFIC ANATOMIC DERANGEMENT

1. Pelviureteric junction obstruction in the Horseshoe Kidney

Pelviureteric junction obstruction is the most common congenital

genitourinary abnormality found in association with a horseshoe kidney, occurring

in approximately in 15% of cases.(72,73) The horseshoe kidneys frequently

demonstrate pyelocaliectasis without obstruction. This may be the result of a mild

resistance to flow where the ureter crosses the isthmus. Once the diagnosis of

functionally significant PUJ obstruction is made, the options for correction are the

same as in normally rotated kidneys. Most dismembered pyeloplasties in

otherwise normal kidneys are performed through an extraperitoneal flank or

posterior approach.

Because of concern, regarding angulation and obstruction at the isthmus,

division of the isthmus and nephropexy to allow more dependent drainage of the

PUJ have been proposed.

Ureterocalicostomy may be considered as an alternative to dismembered

pyeloplaty for repair of even primary PUJ obstruction in horseshoe kidneys. It

permits dependent drainage and avoids any problems related to the isthmus.(74)

Newer minimally invasive techniques have been successfully utilised for

PUJ obstruction in horseshoe kidneys, albeit, in small numbers of patients.

Antegrade endopyelotomy has been used successfully in seven of eight reported

cases.

Laparoscopic pyeloplasty has been successfully applied to horseshoe

kidneys (J.H.Ross,H.Winfield 1997). The horseshoe kidney is particularly

amenable to laparoscopic approach; because the anteriorly placed PUJ is easily

exposed and manipulated with this technique.

2. Pelviureteric junction obstruction in the lower segment of duplex

collecting system.

Vesicoureteric reflux is often seen in the lower segment of a completely

duplicated system and is some times associated with pelviureteric junction

obstruction. The following procedures can be done

1. When the duplication is complete dismembered pyeloplasty may be

performed.

2. When the lower pole ureter of a partially duplicated system is short, the

entire lower pole ureter is excised and a lower to upper pole

pyeloureterostomy is performed.

3. When there is marked hydronephrosis of the lower-pole moiety,

particularly if the pelviureteric junction obstruction is difficult to expose,

then an ureterocalicostomy may be performed.

4. If the lower pole is poorly functioning, a lower-pole heminephrectomy is

appropriate.

5. If a dysplastic upper pole segment is present, owing to an ectopic ureter or

ureterocele, if may be excised at the time of the lower pole repair.

Alternatively, an upper to lower pole ureteropyelostomy may be performed

at the time of the lower pole pyeloplasty.

3. Pelviureteric junction obstruction in the pelvic kidney

Renal ectopy refers to a kidney outside the renal fossa. It is a rare finding,

occurring in 0.01 to 0.08 percent of patients.(75) According Gleason the sites

include pelvic (55%)(Fig No.14), crossed (32%), lumbar (12%), and thoracic

(1%). Approximately half of such kidneys are hydronephrotic. This results from a

variety of causes, including PUJ problems (37%), VUR and lower tract problems

(26%) and hypoplastic adynamic segments at the UVJ (15%). All cases require

lower tract imaging and accurate anatomical upper tract imaging. Surgical

correction requires special considerations. Position, incision, and approach must

be individualized; in a majority a modified Gibson incision allows an

extraperitoneal approach. Open pyeloplasty is usually successful. Experience with

less invasive techniques is limited.

ANTENATALLY DETECTED HYDRONEPHROSIS

The clinical presentation of the pelviureteric obstruction has dramatically

changed since the advent of maternal ultrasonographic screening. Before the

routine fetal ultrasound the commonest presentation was abdominal flank mass.(76)

50% of abdominal masses in newborns are of renal origin with 40% being

secondary to pelviureteric obstruction. The newborns are also present with

abdominal pain, urinary tract infections, irritability, vomiting, and failure to thrive.

Fetal urinary tract dilatation is present approximately 1in 100-200

pregnancies. Male to female ratio is 3: 1 and usually sporadic. Left kidney is

commonly involved.

Associated Anomalies

10% of the PUJ is associated with vesicoureteric reflux. The association of

multicystic dysplastic kidney and contralateral pelviureteric junction obstruction is

well known. The dysplastic kidney reflects the extreme end of the clinical

spectrum of pelviureteric junction obstruction. Bilateral pelviureteric junction

obstruction is 10-36% of cases.(77)

Differential Diagnosis

Dilatation of urinary tract can be secondary to obstructive or nonobstructive

causes. The various obstructive causes include pelviureteric junction obstruction

(44%), VUJ obstruction (21%), multicystic dysplastic kidney, ureterocele/ectopic

ureter, duplicated collecting system (12%), posterior urethral valves (9%) and

hydrometrocolpos.

The non obstructive causes include physiological dilatation, vesicoureteric

reflux (14%), prune belly syndrome, renal cyst and megacalicosis.

Indications for maternal fetal ultrasonography

Whenever the discrepancy in the expected fundal height for gestational

age should undergo prompt an examination to detect multiple fetus,

oligohydromnios and polyhydromnios. A history of previous pregnancies

associated with congenital anomalies is a specific indication for ultrasonographic

screening during pregnancy.(Fig no.1)

A systematic approach to the prenatal diagnosis of urinary tract

abnormalities improves the yield of the ultrasonographic examination. (Schlussel

RN, MandellJ et al) (78)

This includes the following steps

1. Assessment of fetal size and maturity.

2. Assessment of amniotic fluid volume (AFV), which is a semi quantitative

technique. Currently, there are two methods to estimate AFV; amniotic fluid

index (AFI) or measurement of the single deepest vertical pocket of amniotic

fluid. Current ultrasonographic criteria for oligohydramnios (actual AFV <

500 ml) include an AFI of 5.0 cm or less and a two - diameter amniotic fluid

pocket of less than 15 cm. Both methods are poor predictors of

oligohygramnios.

3. Identification of the gender of the fetus.

4. Localisation and characterisation of urinary tract abnormalities (bladder

volume, kidney size, anteroposterior diameter of the renal pelvis, echotexture

of the kidney, and the presence of cortical cysts).

5. Identification of associated abnormalities.

6. Monitoring the detected lesions and their impact on the overall health of the

fetus.

Dilatation has been considered significant when AP diameter of renal

pelvis measured 10mm or more irrespective of gestational age. In addition a ratio

of renal pelvis to AP diameter of kidney > 0.5 has been considered significant.

Grigon et al (79) graded the fetal hydronephrosis into 5 grades as follows

Grade I – detectable renal pelvic dilatation.

Grade II – dilatation greater than 1cm.

Grade III- IV – further degree of pyelectasis with dilatation greater

than1.5cms.

Grade V – association with atropic cortex.

Dilatation of the collecting system can occur in the absence of

obstruction and is termed as physiological hydronephrosis.

Typically, the ureter is of normal caliber and is not seen.(80) But if it

dilated the size of ureter is also assessed ultrasonographically and graded 1-3

according to ureteral size width < 7mm, 7-10mm, >10mm respectively.(81)

Harrison et al suggested that proportion of more than 1:2 of the pyelon

width to the kidney width is pathological and may be diagnosed as fetal

hydronephrosis.(82)

In order to standardize postnatal evaluation of prenatal hydronephrosis, a

grading system of postnatal hydronephrosis was implemented in 1993 by society

for fetal urology.

The Society for fetal urology grading system is as follows

Grade 0 – Normal kidney with no hydronephrosis.

Grade 1 – Slightly dilated renal pelvis without caliectasis.

Grade 2 – Moderately dilated pelvis with mild caliectasis.

Grade 3 – Large renal pelvis, dilated calyces, and normal renal parenchyma.

Grade 4 – Very large renal pelvis, large dilated calyces, with

thinning of the renal parenchyma.

P. A. Dewan et al describe a new sign in ultrasound examination that may

help to identify those fetuses who have high intrarenal pressure and therefore

justify more aggressive management, while obviating the need for intervention for

those in whom it is not present. The egg-shell sign consists of a thin crescent of

increased echogenicity over a distended calyx and, in this case, was documented to

be associated with other features of raised intrarenal pressure.(101)

AMNIOCENTISIS AND ASSESSMENT OF FETAL URINE

FUNCTION

This is an invasive procedure to be done only by experienced personal in

patients in whom renal ultasound has revealed several problems like bilateral

hydronephrosis or silidary kidney, progression of severity, oligohydromnios or

absence of corticomedullary junction differentiation, which suggest bilateral

hydronephrosis and dysplasia of the kidneys.

The goal is to identify fetuses that are risk while in utero, of total renal

destruction and pulmonary hypoplasia when there is a reasonable hope of

beneficial treatment. This test currently carries of two types.

1. Evaluation of amniotic fluid fetal urine to assess fetal tubular function.

2. Evaluation of proteins in fetal serum or urine to assess

fetal glomerular filtration rate.

Between 16 and 21 weeks of gestation, the fetal urine normally becomes

progressively more hypotonic because of selective tubular reabsorption of sodium

and chloride in excess of free water. The most quoted values for fetal urinary

electrolytes and osmolatity abnormalities indicative of an impaired renal function

in the fetus with detectable upper tract dilatation are as follows

1. Osmolality of less than 210mosm/l

2. Urinary sodium concentation of less than 100 meq/l

3. Urinary chloride concentration of less than 90meq/l

4. Urinary output of less than 2ml/hr

5. Urinary calcium levels greater than 8mg/dl (normal <8mg/dl) are the most

sensitive indication of renal dyssplasia(100%) but have a demonstrated

specificity of only 60%.

6. Beta 2 microglobulin elevation in fetal urine of greater than 4mg/l is

pathological.

Management

Antenatal Management

Fetuses with unilateral dilatation of upper tracts and a normal contralateral

kidney are simply observed with serial ulrasound examinations at 1-2 weeks

intervals till term.

Fetuses with bilateral hydronephrosis who are at higher risk for obstructive

uropathy and in whom the Amniotic fluid index should be carefully monitored.

Once the oligohydromnios develops, fetal urine sampling is the next step to be

evaluated.

Once a fetus with salvageable function is selected, early delivery is

considered if it is more than 32 weeks of gestation. If it is between 20-32 weeks

various fetal interventions are considered. It is less than 20 weeks with

oligohydramnios termination of pregnancy to be considered.(96)

Scheme of management for antenatal diagnosed bilateral hydronephrosis

Bilateral hydronephrosis

Normal amniotic fluid volume oligohydramnios ↓ Detailed sonography and Fetal urine analysis ↓ Ranal dysplasia Poor renal function Reasonable renal function Serial monitoring and termination of ↓ ↓ Postnatal management pregnancy Mature lung immature lungs ↓ ↓ Early delivery in utero decopression

Postnatal evaluation and Management

All the newborns with a prenatal diagnosis should undergo physical

examination at birth to rule out associated anomalies. Prenatally detected

hydronephrosis should be confirmed postnatally on day 2 or 3 of life(Fig no

2),because neonates may have transient oliguria and dilated obstructed collecting

system may appear for 24-48 hours of life.(83) The ultrasound evaluates pelvic

dilatation, which is graded according to the SFU grading system. Ureteric

dilatation is specifically looked for. The bladder is evaluated for size, wall

thickness, presence of diverticulum or ureterocele and dilatation of the posterior

urethra.

Next VCUG should be performed to rule out posterior urethral valve, a

bladder diverticulum, or vesicoureteric reflux. Even if the ultrasound is normal a

VCUG should be performed, because reflux may be the cause of fetal

hydronephrosis.

If the ultrasound and VCUG are normal, then only a follow up ultrasound

in 6-8 weeks is necessary.

If the postnatal sonogram shows grade 1 or 2 hydronephrosis and the

VCUG is normal, then the pelvicalyceal dilatation is due to physiological. These

children should follow with ultrasonogram in 3-6 months period.

If the sonogram shows grade 3 or 4 hydronephrosis and there is no reflux,

the upper tract must be evaluated further with T99 MAG 3 or DTPA diuretic

renogram at 4-6 weeks of age.

The goal of early evaluation is to determine whether a true anatomic

obstruction is present that should be repaired or whether it is safe to follow the

infant nonoperatively.

Some authors advocate early surgical intervention to prevent damage to

matured nephrons. While others feel that early surgery carries no specific benefit.

Algorithm for the the postnatal evaluation of infants with prenatally detected hydronephrosis Prenatal diagnosis of hydronephrosis ↓ Depending on gestational age, assessment of pulmonary function Postnatal ultrasound at 3-5 days Normal Abnormal ↓ ↓ VCUG VCUG ↓ ↓ Normal vesicoureteric reflux Normal ↓ ↓ ↓ Ultrasound at 3 months Prophylactic antibiotics Diuretic renal scan ↓ ↓ <40% Differential function >40% Differential function Delayed drainage ↓ ↓ Surgery ? Prophylactic antibiotics Diuretic renogram & ultrasound at 3-6 months Koff and Campbell suggested that immediate postnatal surgical

intervention is unnecessary in the majority of newborn child with pelviureteric

junction obstruction. These babies should be followed up several examinations to

observe anatomical and functional improvement. Surgery is undertaken in infant

with deteriorating renal function.(84,85)

Follow up ultrasound may be performed 3-6 months after operation when

maximum improvement can be seen. Radionuclide scan are useful to monitor the

post pyeloplasty function and drainage.

Bilateral Hydronephrosis

If the ultrasound finding is suggestive of posterior urethral valve, an early

voiding cystourethrography is performed to confirm the diagnosis and treat

accordingly. All male babies with bilateral hydronephrosis are evaluated with

voiding cystourethrography to rule out bladder outlet obstruction and reflux. In

female babies direct radionuclide cystography is considered only if ureter is

dilated on ultrasound.

In bilateral pelviureteric obstruction the symptomatic side or the side with

better function should be operated first. If nephrectomy is considered on one side,

the pyeloplasty should precede this.

Unilateral Hydronephrosis

The work up depends on the status of the opposite kidney on ultrasound. If

the opposite kidney is normal, and the ureter is not seen on ultrasound, the most

probable diagnosis is pelviureteric junction obstruction and the baby only requires

a diuretic renogram at 4-6 weeks of age for further evaluation.

If the opposite kidney shows multicystic dysplastic kidney, voiding

cystourethrography should be done to rule out vesicoureteric reflux. If it is present,

aggressive management should be taken to save the single functioning kidney.

At least 35 to 50% of antenatally detected hydronephrosis reveals an

apparent obstruction at the pelviureteric junction.

Two schools of management have been emerged. Early intervention is

supported by the fact that the functional recovery is better with early relief of

obstruction and there is a significant risk of irreversible functional loss while on

observation alone. Where as, delayed intervention until the obstruction is proved

on serial assessment is lead onto renal damage. Recent evidence suggested that

early pyeloplasty improved the renal growth and function. Diuretic renography is

gold standard to diagnose obstruction. This should be done after 4-6 weeks of age.

Ransley et al proposed treatment protocols based on the split renal function

on the first diuretic renogram done after 4 weeks of age. Kidneys with SRF >

40% are observed and SRF <20% undergo operation or percutaneous drainage.

Kidneys with SRF 20-39% undergo repeat scan at 3 months of age and those

kidneys with SRF <40% then undergo pyeloplaty while the kidneys that improved

good function are observed.(85)

COMPLICATIONS OF PYELOPLASTY

1. Persistent Urinary Leakage

If the persistent urinary leakage occurs beyond 10 days, evaluation with

Ultrasonogram and antegrade or retrograde pyelography should be entertained.

Conservative measures may preserve the repair but if hydronephrosis persist,

reexploration should be considered.

2. Delayed Opening of Anastomosis

This is usually indicated by increased nephrostomy drainage following

removal of a splint or in patients without splint, by persistent large volume of

drainage. It is caused by postoperative edema at suture line or by blood clot. If the

persistent high volume drainage continues, appropriate measures should be

undertaken to evaluate the patency of the anastomosis.

3. Urinoma Formation

Collection of urine around kidney caused by anastomotic leakage or may

follow the removal of a nephrostomy tube. In most cases it results from

inadequate placement or early drain removal. The most frequent cause of an

urinoma seems to result from premature removal of the flank drain in the absence

of nephrostomy. The presence of urinoma should be suspected in a patient who

suddenly ceases urinary leakage an opposed to those who have a gradual

diminution and cessation of drainage. Should an urinoma be suspected, ultrasound

is helpful to ascertain diagnosis. Many small urinomas resolve spontaneously

while other must be drained surgically.

4. Infection, Anastomotic Disruption and Stone Formation

In pyeloplasty if the urine becomes infected with E.coli, proteus the

absorbable sutures may either become a nidus for stone formation or reabsorb too

quickly, causing leakage or disruption of the suture line. The faulty suture

technique or vascular compromises, the presence of infected urine are the other

factors responsible for anastomotic disruption. Prevention and treatment of the

infection may improve the outcome.

5. Hemorrhage

In most cases it is related to the presence of a nephrostomy. It can be

controlled with hydration and transfusion.

6. Ureteral Kinking or Angulation

This complication occurs when the kidney is retuned to the renal fossa,

which can alter the relationship of the renal pelvis to the newly anastomosed

ureter. To prevent this occurrence, Hendren suggest pexing the kidney to the

psoas muscle.

7. Anastomotic Fibrosis and Stenosis

It may be as a result of vascular compromise, persistent urinary leakage,

extravasation of infected urine, or any combination thereof. By keeping these

events to a minimum, the incidence of anastomotic failure will be reduced.

8. Persistent Hydronephrosis and Secondary Nephrectomy

The term persistent hydronephrosis should probably be replaced by

radiological deterioration. This may be occurring in a small group of patients.

Preservation of the ureteral vasculature, a watertight anastomosis,

prevention of infection and care the placement and management of drains enhance

the opportunity for a favorable outcome.

REDO PYELOPLASTY

The failure rate of pyeloplasty is 5 %.(86) The causes of failed pyeloplasty

after allowing 3 months waiting period to allow for anastomotic edema to settle

are

1. Dense scarring around the anastomosis in all cases.

2. A high non dependent anastomosis in some cases.

The anastomosis is however almost always proved patent. The

points that need attention during pyeloplasty are (87)

1. Meticulous technique avoiding uerteral ischemia.

2. Avoid an undrained urinary extravasation

Both are chief causes of perianastomotic scarring .A well placed drain

close to the pyeloplasty is essential. The use of nephrostomy also reduces the

chance of urinoma and associated with extremely low redo rate.

Indications for redo pyeloplasty

1. Recurrence of palpable lump and pain

2. Un resolving urosepsis

3. Lack of drinage in nephrostogram if nephrostomy is in place.

While a successful pyeloplasty should result in diminishing renal dilatation

in ultrasound and improving renal function in radionuclide scan, the following

points need to be remembered.

1. The ultrasound scan show increased dilatation in the first few months

after pyeloplasty, which then resolves gradually.(88)

2. The function on radionuclide study remains unchanged in many

pyeloplasties and can even show some deterioration in 7% of

successful pyeloplasties.(89)

MANAGEMENT OF FAILED PYELOPLASTY

Surgery for failed pyeloplasty should not be considered for at least 2

months. There are five basic approaches to the treatment of secondary PUJ

obstruction are (90)

1. Redopyeloplasty

2. Ureterocalicostomy

3. Laparoscopy

4. Endopyelotomy

5. Nephrectomy

Open procedure

The principles of open redo pyeloplasty are excision of the scarred

reanastomosis in tension free dependent position and routine use of stent and

nephrostomy.

When tensions free anastomosis in not possible, the lower calyx denuded

of overlying renal parenchyma, and anastomosed to the spatulated ureter as a

ureterocalicostomy.

Endourological procedure

Endopyelotomy can give satisfactory results in failed pyeloplasty cases

provided

1. The stenotic segment is not long

2. The kidney is not very hydronephrotic

3. In older children

4. There is no vessels lateral or posterolateral to the PUJ where the

incision will be made.

5. The stenotic PUJ can be cannulated.

The results of endopyelotomy for failed pyeloplasty (100%) seem to be

better than endopyelotomy done as a primary procedure (62%) for children.(91)

6. RESULTS OF STUDY AND DISCUSSION

In this study 41 cases of the pelviureteric junction obstruction were studied

from the period of July 2003 to September 2005.

Distribution of Cases

Among the 41 cases unilateral 38 cases, bilateral 3 cases.

SIDE NO OF CASES PERCENTAGE

UNILATERAL 38 93%

BILATERAL 3 7%

38

3

UNILATERAL BILATERAL

Among the 38 cases of unilateral, 13 (32%) were on right side and 25 were

on left (61%) and 3 cases (7%) were having bilateral obstruction. This is

DISTRIBUTION OF CASES

consistent with observation of Johnston JH. J Urol 1977:117:97& Snyder.Urol

Clin North Am 1980:7; 273.

SIDE NO OF

CASES PERCENTAGE

RIGHT 13 32%

LEFT 25 61%

BILATERAL 3 7%

Age and Sex Incidence

Age

In this series commonest age group is 6-12years. 16 cases (39%) are in this

age group. Only 3 cases presented with antenatal diagnosis.

AGE MALE FEMALE TOTAL PERCENTAGE

0-1Y 3 2 5 12%

1-3Y 9 2 11 27%

3-6Y 6 3 9 22%

6-12Y 10 6 16 39%

AGE INCIDENCE

32

9

2

6

3

10

6

0123456789

10

0-1Y 1-3Y 3-6Y 6-12Y

MALE FEMALE

Sex Ratio

In this series Boys were commonly affected than girls.. Male to female

ratio is 2:1. Of 41 cases studied 28 cases are male (68%) and13 cases are female

(32%). Williams and Karlaftis, 1966, Kelalis et al 1971 also observed the same

incidence.

Clinical Presentation

Before the advent of antenatal ultrasonography, most infants with

pelviureteric junction obstruction present with abdominal masses ,Urinary tract

infection, Hematuria, and gastrointestinal discomfort.

Most of the cases now are being detected during antenatal ultrasonographic

screening. But in this study only 3 cases presented with antenatal diagnosis.

In this series the main presenting symptom was mass per abdomen.

(Fig No.16&17). 10 presented with mass and pain abdomen. 11 cases presented

with only urinary tract infections. Most of the patients had associated

gastrointestinal symptoms like abdominal discomfort,nausea,vomiting. Hematuria

occurred in only 2 cases.

Associated Anomalies

In this series 6 of the patients had other congenital anomalies namely

1. Malrotation

2. Hemivertebra

3. Situs inversus with dextrocardia

4. Unilateral renal agenesis (Fig No:8&9)

5. Glanular hypospadias

6. Right undescended testis

Pelviureteric junction obstruction in Other Anomalies of Kidney

In this series pelviureteric junction obstruction occurred in an ectopic

kidney (left pelvic kidney) (Fig No.12&14) and another occurred in crossed

ectopia.(Fig No.16)

Etiology

Among the 41 cases studied, 37 had intrinsic cause for the obstruction. The

length of the narrowed segment was ranging from 0.5cm to 2cm in length. There

was one case with polar vessel. In this series pelviureteric junction obstruction was

present in a dysplastic kidney for which nephrectomy was done. All the excised

specimen were sent for histopathological examination.

TREATMENT

Conservative Management

In this series only 3 patients were treated conservatively. Of 3, 2 were

diagnosed antenatally.

Surgical Treatment

In 37 patients 39 Anderson Hynes pyeloplasty was done including 3

bilateral cases. Of these

Nephrostomy tube + Stent + Drainage tube in 4 cases,

Stent with drainage tube in 19 cases,

Nephrostomy tube with drainage tube in 1 case,

Drainage tube alone kept in 13 cases.

Nephrectomy was done in 1 case, in which the kidney was dysplastic.

In this series the transanastomotic stent was removed after 48 hours,

followed by the removable of nephrostomy tube on the 5th postoperative day. The

extraperitoneal drain was removed on 8 th PO day.

Post Operative Complications

Mild urinary leak occurred through drainage site in most of the cases,

which subsided slowly over a period of 7 to 10 days. One patient developed

perinephric urinoma which was treated by percutaneous drainage. Another had

intestinal obstruction in the postoperative period. On laparotomy a loop of small

bowel was found to herniate through the ureteric mesentery and gangrenous. He

also had malrotation. Resection of the gangrenous bowel loop along with Ladds’

procedure was done.

Outcome

Of 39 Anderson Hynes pyeloplasties done, the success rate was 95 % in

this series.

Follow up Study

All the patients were followed over a period of one month to two years.

During the follow up most of cases were examined clinically and radiologically

with ultrasound and few cases with intravenous urogram and isotope renogram .In

the ultrasound examination the pelvicaliceal dilatation and ureter caliber were

assessed(Fig No. ). The size of the kidney was found to be without much

alteration. In postoperative intravenous pyelogram, dye was seen in early pictures

which were not present in preoperative intravenous pyelogram(Fig No. 21)

CONCLUSION

The incidence and presentation in this study are consistent with the

most of the reports given in the literatures. Eventhough the other series show the

incidence of antenatally diagnosed hydronephrosis is more, in this series ironically

only 3 cases had antenatally diagnosed hydronephrosis. The results of the

dismembered pyeloplasty (Anderson Hynes pyeloplasty) are comparable with any

other reports in the literature. In conclusion the Anderson Hynes pyeloplasty is the

most preferred method to treat pelviureteric junction obstruction.

PROFORMA FOR PELVIURETERIC JUNCTION OBSTRUCTION

IN CHILDREN

Name of the patient: IP No : Age : sex: DOA: DOD : Diagnosis : PRESENT HISTORY

1. abdominal mass 2. abdominal pain 3. Hematuria 4. urinary tract infection – dysuria pyuria 5. gastrointestinal symptoms – nausea Vomiting 6. Antenatally diagnosed 7. Hypertension

Side Right Left Bilateral PAST HISTORY Previous surgery H/o Trauma FAMILY HISTORY ANTENATAL HISTORY POSTNATAL HISTORY INVESTIGATIONS: Urine- culture and sensitivity Blood – urea - creatinine

1. Ultrasonogram 2. Intravenous pyelogram 3. Micturiting cystourethrogram 4. Diuretic renogram 5. CT scan

TREATMENT

1. Conservative management 2. Pyeloplasty a) Anderson Hynes pyeloplasty with nephrostomy tube and drain with nephrostomy and stent and drain with stent alone and drain with extraperitoneal drain alone b)Foley Y plasty c)Spiral flap procedure d) Vertical Flap procedure e)Ureterocalycostomy

3. Pyeloplasty for UPJ obstruction in 1.Horseshoe kidney 2.Lower segment of duplex system 3. Pelvic kidney 4. Laparoscopic dismembered pyeloplasty

5. Nephrectomy

Postopertive complications Persistent urinary leakage Delayed opening of anastomosis

Formation of urinoma Infection Anastomotic disruption Stone formation Hemorrhage Ureteral kinking or angulation Anastomotic fibrosis Persistent hydronephrosis Secondary nephrectomy Follow up

MASTER CHART

S no

NAME OF THE PATIENT

AGE SEX

IP NO

DIAGNOSIS

ANT R L BIL CON

SURGERY

N+S+D S+D DT N+D

NE M M+P

UTI GIT

1 Ranjithkumar 1 ½Y M 214712 UPJ + AHP + + 2 Jesimabanu 8 y F 226638 UPJ + AHP + + 3 Meenakshi 3 ¼ y F 239805 UPJ + AHP + + 4 Selsia 1 ½ y F 239383 HN

with atrophied kidney

+ AHP + +

5 Nazeema Banu 4/12y F 346535 UPJ + + + + 6 Muthusamy 10 y M 201810 UPJ + AHP + + + 7 Manimala 11 y F 248910 UPJ + AHP + + + 8 Rajapandi 11 y M 253782 UPJ + AHP + + 9 Prasanth 9 y M 255676 UPJ + AHP + + 10 Duraipandi 7 y M 258909 UPJ + AHP + + + 11 Vivek 11 y M 269410 UPJ + AHP + + 12 Nagaraj 3 y M 271120 UPJ + AHP + + 13 Lakshmipathy 5 y M 321052 UPJ

with crossed ectopia

+ AHP + +

14 Suriya 10 y F 275779 UPJ + Bil. AHP + + + 15 Devadarshini 7 y F 289417 UPJ + AHP + 16 Pandi 3 y M 287948 UPJ + AHP + + + 17 Sathiyapriya 11 y F 296247 UPJ + AHP + 18 Arun 2 y M 308836 UPJ + AHP + + 19 Vignesh 1 y M 311713 UPJ + AHP + + 20 Pavithra 5 /12 F 314231 UPJ + AHP + + 21 Sivapriya 9y F 315550 UPJ + AHP + + 22 Aravinth 7 y M 326788 UPJ + AHP + + 23 Sivanath 12 y M 231207 UPJ + AHP +

24 Meena 6 y F 339432 UPJ + R-AHP + 25 Buvaneswari 3 y F 342682 UPJ + AHP + + 26 Ajithpandi 5 y M 350619 UPJ + AHP + + 27 Sivasankar 4 y M 351736 UPJ in

single kidney

+ AHP + + +

28 Balamurugan 1 ½ y M 353547 UPJ + AHP + + 29 Sivanesan 1 ½Y M 360657 UPJ + AHP + + + 30 Naveenkumar 11 M 360664 UPJ + AHP + + + 31 Praveen 6 y M 364475 UPJ + + AHP + 32 Nagarajan 7 y M 365605 UPJ + R-AHP

+

33 Aswin 5 y M 369265 UPJ + AHP + + 34 Muthukumar 1 y M 369472 UPJ + AHP + + 35 Veerapathiran 11 y M 373142 UPJ + AHP + + + 36 Muthukumaran 11 y M 381779 UPJ + AHP + + 37 Abdulsamed 3 Y M 384317 UPJ + AHP + + 38 Chandru 4 y M 384321 UPJ + AHP + + + + 39 Periyakaruppan 1 1/4y M 267235 UPJ - + AHP + + 40 B/O

guruvammal 6/365 F 380448 UPJ + + + +

41 Ismail 5y M 386471 L-pelvic kidney +UPJ

+ + +

L- left side R- right side BIL- Bilateral CON- Conservative management SUR- Surgery, AHP-Anderson Hynes Pyeloplasty N+S+D- Nephrostomy tube + Stent + Drainage tube S+D - Stent + Drainage Tube D – Drainage tube alone NEPH- Nephrectomy M- Mass M+P – Mass + Pain UTI – Urinary tract infection GIT- Gastrointestinal symptoms

1

REFERENCES

1. Ruano-Gil D, Coca-Payeras A, Tejedo-Mateu A: Obstruction and normal

recanalization of the ureter in the human embryo: Its relation to congenital ureteric

obstruction. Eur Urol 1975;1:287–293

2. BakerLA, GomezRA: Embryonic development of the ureter: acquisition of smooth

muscle (abstract A 1715).J Am Soc Nephrol 1996; 7:1593.

3. Alcaraz A,et al: obstruction and recanalisation of the ureter during embryonic

development. J Urol 145:410, 1991.

4. MoritaT, IshizukaG, TsuchidaS: Initiation and propagation of stimulus from the

renal pelvic pacemaker in pig kidney. Invest Urol 19:157, 1981.

5. Gobe CG,Alexlsen RA: Genesis of renal tubular atrophy in experimental

hydonephrosis in the rat.Role of apoptosis,Lab invest 56:273,1987.

6. Connor J et al: SGP-2 expression as a genetic marker of progressive cellular

pathology in experimental hydronephrosis,Kidney Int 39:1098,1991.

7. Okegawa T et al: Metabolic and cellular alterations underlying the exaggerated

renal prostaglandin and thromboxane systhesis in ureter obstruction in rabbits.

Inflammatory response involving fibroblasts and mononuclear cells, J.Clin Invest

71:81, 1983.

8. Haralambous – Gasser A et al: Collagen studies in newborn rat kidneys with

incomplete ureteric obstruction, Kidney Int 44:593, 1993.

9. Mollard P, Braun P: Primary ureterocalicostomy for severe hydronephrosis in

children, J.Pediatr Surg 15:87, 1980.

2

10. Glick PL et al: Correction of congenital hydronephrosis in utero III. Early mid-

trimester ureteral obstruction produces renal dysplasia, J.Pediatr Surg 18:681, 1983.

11. Gonzales R et al: Early bladder outlet obstruction in fetal lambs induces renal

dysplasia and the prune-belly syndrome, J Pediatr Surg 25:342, 1990.

12. Beck AD: The effect of intra-uterine urinary obstruction upon the development of

the fetal kidney, J Urol 105:784, 1971.

13. Harrison MR et al: Correction of congenital hydronephrosis in utero

decompression reverses the effects of obstruction on the fetal lung and urinary tract, J

Pediatr Surg 17:965, 1982.

14. Lanzone JA et al: Renal hemodynamics in acute unilateral obstruction:

contribution of endothelium-derived relaxing factor, J Urol 153:2055, 1995.

15.Felsen D et al: Involvement of platelet activating factor and thromboxane A2 in the

renal response to unilateral obstruction, J Urol 144:141, 1990.

16.Vari RC , Boineau FG, Lewy JE: Angiotensin or thromboxane receptor antagonism

in rats with congenital hydronephrosis, J Am Soc Nephrol 3:1522, 1993.

17.Cestonaro G et al: Renal lactate dehydrogenase(LDH) isoenzyme pattern in short-

term experimental obstructive nephropathy, Invest urol 17:46, 1979.

18.Bellinger MF et al: Renal function in the fetal lamb: a chronic model to study

effects of ureteral ligation and deligation, J Urol 136:225, 1986.

19.Djurhus JC, Jorgensen TM, Norgard JP: Contrast perfusion provocation in

idiopathic hydronephrosis, Urology 19:611, 1982.

20. Johnston JH et al: Pelvic hydronephrosis in children: a review of 219 personal

cases, J Urol 117:97, 1977.

3

21. Koff SA: Pathophysiology of ureteropelvic junction obstructions. Urol Clin North

Am 1990;17:263–272

22. Koff SA, Hayden LJ, Cirulli C, Shore R: Pathophysiology of ureteropelvic

junction obstruction: Experimental and clinical observations. J Urol 1986; 136:336–

338.7

23. Josephson S: Experimental obstructive hydronephrosis in new-born rats. III. Long-

term effects on renal function, J Urol 129:396, 1983.

24.Murnaghan GF: The dynamics of the renal pelvis and ureter with reference to

congenital hydronephrosis. Br J Urol 1958;30:321–324.

25.Tainio H ,kylmalaT,Heikkinen A. peptidergic innervation of the normal and

obstructed human pyeloureteral junctions. Urol Int 1992;48:31-4

26.Hanna MK: Some observations on congenital ureteropelvic junction obstruction.

Urology 12:151,1978

27. Stephens FD: Ureterovascular hydronephrosis and the "aberrant" renal vessels. J

Urol 1982;128:984–987.

28.Felix W: development of urogenital organs. In KeibelF,HailFP(eds): Manual of

Human Embryology. Philadelphia,JB Lippincott,Vol 2.1912,pp752-979.

29. Williams DI, Karlaftis CM: Hydronephrosis due to pelviureteric obstruction in the

newborn. Br J Urol 1966;38:138–144.

30. Flashner SC, Lower RK: Ureteropelvic junction. In Kelalispp, King LR, Belman

AB editors: Clinical pediatric urology, ed 3, Philadelphia, 1992, WB Saunders.

31.ValayerJ AddaG: hydronephrosis due to PUJ obstruction ininfancy.BrJUro

l54:451-454,1982.

4

32.Dietl: Nerki wedrujace I ich uwiezgnienie.Przegl Lek (Krakow) 3;225,1864.

33.Dhillon HK: prenatally diagnosed hydronephrosis; the Great Ormond street

Experience: Br J Urol 1998;81(2):39-44

34.Ransley PG,Dhillon HK,Gordon I et al: the postnatal

managementofprenatalultrasound.

35. Koff SA, Peller PA, Young DC, Pollifrome DL: The assessment of obstruction in

the newborn with unilateral hydronephrosis by measuring the size of the opposite

kidney. J Urol 1994; 152:596–599

36. Kelfer MS, Korsvik h,Weiss RM,et al; Resistive index ratios in the US

differentiation of unilateral obstructive vs nonobstructive hydronephrosis in

children.pediatr radiol 21:462,1991

37. Claesson G, Josephson S,Robertson B: Experimental partial ureteric obstruction in

newborn rats. VII. Are the long term effects on renal morphology avoided by release

of the obstruction? J Urol 136:1330, 1986.

38. Horowitz M, gershbein AB,Glassberg KI: Vesicoureteral reflux in infants with

prenatal hydronephrosis confirmed at birth: racial differences.J urol 1999;161:24

39. Mesrobian HGJ, Kelalis pp: Ureterocalicostomy: indications and results in 21

patients, J Urol 142:1285, 1989.

40.KelalisP ,King R WBSounders company

41. KassEJ, Fink-Bennett D:Contemporary techniques for the radioisotopic evaluation

of the dilated urinary tract.J urol clin North Am 1990;17:273

42. Homsy YL,Mehta PH,Hout D, et al: Intermittent Hydronephrosis: A diagnostic

challenge. J Urol 140:1222-1226,1988

5

43. O’Reilly PH: Diuresis renography:Recent advances and recommended

protocols.Br J Urol 69:113-120,1992

44. O’ Reilly PH, Testa HJ,Lawson RS,et al: diuresis renography in equivocal urinary

tract obstruction.Br J Urol 50:76-80,1978.

45. The society for fetal urology and members of the pediatric nuclear medicine

council of the society of Nuclear medicine: the Well Tempered diuretic renogram: A

standard method to examine the asymptomatic neonate with hydronephrosis or

hydroureteronephrosis.J Nucl Med 33:2047-2051,1992.

46. ConwayJJ: well tempered diuresis renography: its historical development,

physiological and technical pitfalls, and standardized technique protocol. Semin Nucl

Med 22:74-84,1992

47. Bagley DH,LiuJB,Goldberg BB: Endoluminal sonographic imaging of the

ureteropelvic junction. Endourol 1996;30:400-402,1996.

48. Whitaker RH: Clinical assessment of pelvis and ureteral

function.Urology1978;12:146–150.

49. Elder JS: Experimental studies of partial ureteral obstruction, Dial pediatric urol

14:2,1991

50. Fung LCT,Atala A: Constant elevation in renal pelvic pressure induces an increase

in urinary N acetyl beta D glucosaminidase in a nonobstructive porcine model.J Urol

1998;159:212

51. Granaliano G,GesualdoI, Bartoli F,et al: MCP-1 and EGF renal expression and

urinary excretion in human congenital obstructive nephropathy. Kidney Int

2000;58:182.

6

52. Foley FEB: New plastic operation for strictures at UPJ: report of 20 operations. J

urol 38:643-672,1937.

53. Anderson JC, Hynes W: Retrocaval ureter: A case diagnosed preoperatively and

treated successfully by a plastic operation. Br J Urol 1949;21:209.

54. GonzalezR, Aliabadi H: Posterior lumbotomy in pediatric pyeloplasty. J urol

137:468-470,1987.

55. Culp OS, DeWeerd JH: A pelvic flap operation for certain types of ureteropelvic

obstruction: Preliminary report. Mayo Clinic Proc 1951;26:483.

56. Scardino PL, Prince CL: Vertical flap ureteropelvioplasty: Preliminary report.

South Med J 1953;46:325.

57. Hawthorne NJ, Zincke H, Kelalis P.P; Ureterocalicostomy an alternative to

nephrectomy. J Urol 115:583, 1976.

58.Bassiouny IE.Salvage pyeloplasty in non visualizing hydronephrotic kidney

secondary to ureteropelvic junction obstruction.J urol 1992;148:685-

59.Clayman RV,Kavoussi LR: Endosurgical techniques for noncalculous disease. In

campbell’s urology. Philadelphia, WB Saunders, 1992,p 2306.

60. Davis DM: Intubated ureterotomy: A new operation for ureteral and ureteropelvic

stricture. Surg Gynecol Obstet 1943;76:513.

61.Badlani G,Karlin G, Smith AD: Complications of endopyelotomy: Analysis in

series of 64 patients J Urol 140:473-475,1988

62. Karlin GS,Badlani GH, Smith AD: Endopyelotomy versus open pyeloplasty:

Comparison in 88 patients. J urol 140:476-478,1988.

7

63. Brooks JD, Kavoussi LR,Preminger GM et al: comparision of open and

endourological approaches to the obstructed ureteropelvic junction. Urology

1995,46:791-795.

64. Van Cangh PJ, Wilmart JF, OpsomerRJ et al: Longterm results and late recurrence

after endoureteropyelotomy: A critical analysis of prognostic factors. Urology

151:937-937,1994

65.Preminger GM, Clayman RV,Nakada SY, et al: A multicenter clinical trial

investigating the use of a fluroscopically controlled cutting balloon catheter for the

management of ureteral and ureteropelvic junction obstruction. J urol 157:1625-

1629,1997.

66. Tan HL, Roberts JP, Grattan-Smith D: Retrograde balloon dilation of ureteropelvic

obstructions in infants and children: Early results. Urology 1995;46:89–91.

67. Figenshau RS, Clayman RV,Colberg Jw, et al: Pediatric endopyelotomy: the

Washington university experience. J urol 156:2025-2030,1996

68. Schuessler WW, Grune MT, Tecuanhuey LV, Preminger GM: Laparoscopic

dismembered pyeloplasty. J Urol 1993;150:1795–1799.

69. Peters CA, Schlussel RN,Retik AB: pediatric laparoscopic dismembered

pyeloplasty. J urol 153:1962,1995.

70. Hubert J: Robotic pyeloplasty. Curr urol Rep 2003;16:189

71. MunvarR,Del PizzoJJ,Sosa RE, Poppas DP: Minimally invasive surgical

management of ureteropelvic junction obstruction: Laparoscopic and robot-assisted

laparoscopic pyeloplasty. J Long term Effects Med Implants 2003;13:367

72.Das S, Amar D: Ureteropelvic junction obstruction with associated renal

anomalies. J urol 131:872-874,1984.

8

73. Pittis WRJr, Muecke EC: Horseshoe kidneys: A 40 year experience. J urol

113:743-746,1975

74. MesrobianHJ,Kelalis PP: Ureterocalicostomy: Indications and results in 21

patients. J urol 142:1285-1287,1989.

75.Chelimsky CG,GonzalezR: abnormalities of the kidney. In o’Donnell B, Koff SA:

Pediatric urology.Butterworths,1997.

76. Johnston JH, Evans JP, Glassberg KI, Shapiro SR: Pelvic hydronephrosis in

children: A review of 219 personal cases. J Urol 1977;117:97–101.

77. Snyder HM III, Lebowitz RL, Colodny AH, et al: Ureteropelvic junction

obstruction in children. Urol Clin North Am 1980;7:273–290.

78. schlussel RN,Mandell J: Antenatal intervention. In Libertino JA: Reconstructive

urologic surgery.St. Louis,Mosby,1997,pp 603-609

79. Grignon A, Filion R, Filiatrault B, Robitaille p,homsy Y,Boutin H, Leblond R.

Urinary tract dilatation in utero, classifications and clinical applications. Radiol

1986;160:645-7

80. Shackelford GD,Kees-FoltsW,Cole BR. Imaging the urinary tract. Clin in perinatol

1992;19:85-119

81.Fernloach SK, MaizelsM,ConwayJJ. Ultrasound grading of hydronephrosis:

introduction to the system used by the society for fetal urology.pediatric radiol

1993;23:278-80

82. Harrison et al Mr,Filly RA. The fetus with obstructive uropathy.Pathophysiology,

Natural History,selection and treatment in the unborn patient:Prenatal diagnosis and

treatment. Philadelphia: WB Saunders, 1990:238-402

9

83. DejterSWJr,Gibbons MD. The fate of infant kidneys with fetal hydronephrosis but

initially normal postnatal sonography.J urol 1989;142:661

84. Ransley PG,DhillonHK, GordonI,Duffy pG,Dillon MJ,BarrettTM. The postnatal

management of hydronephrosis diagnosed by prenatal ultrasound.J urol1990;144;584-

7

85. KoffSA,Campbell K. Nonoperative management of unilateral neonatal

hydronephrosis. J urol 1992;148:525-31

86. ureteropelvic junction obstruction in urologic surgery in infants and children(1999)

Lowell R.King WB Saunders company. Philadelphia

87. The operative management of recurrent ureteropelvic junction obstruction 1997.

Rohrmann D. Snyder HM, Puckett JW et al. J.Urol, 158, 1257-1259.

88. Sonographic evaluation of PUJ after pyeloplasty 1996. L.R.King et al J.Urol. 156,

2020-2024.

89. Analysis of outcome of 203 pyeloplasties in infancy 2000. Horben, Wischerman,

Borne et al.

90. Ellsworth PI,Walker RD, ElderJS: management of the failed Pyeloplasty. AUA

Update series 1997;16(lesson):130

91. Pediatric Endopyelotomy- the Washington University Experience 1996. J.Urol

156, 2025-2030.

92. Textbook of paediatric surgery – by James A. O’Neill Jr, Marci. Rowe,

JayL.Grosfeld, EricW.Fonkalsrud, Arnold G. Coran.

93. Kelalis clinical paediatric urology.

94. Campbell’s urology.

95. Paediatric urology by king.

96. Essentials of paediatric urology by David FM Thomas.

97. Textbook of Neonatal surgery by D. K. Gupta.

10

98. Pediatric urology by JohnP.Gearhart,W.B>saunders company,Philadelphia

99. The urologic clinics of North America25; 2:1998

100. Jenetschek G,Peschel R,Altarac S,et al: Laparoscopic and retroperitoneoscopic

repair of ureteropelvic junction obstruction.Urology 47:311,1996.

101. P. A. Dewan, K. Anderson, P. A. Dewan Pediatric surgery international Vol

16,No 7,2000.

ANTENATALLY DETECTED RIGHT HYDRONEPHROSIS

1 2 3 FIG NO- 1 - Antenatal USG shows increased pelvic AP diameter of right kidney FIG NO- 2- Postnatal USG at 48 hr shows AP diameter of the pelvis is 1.7cm in right kidney FIG NO-3 - The contralateral kidney of the same patient showed normal kidney ULTRASONOGRAM OF PELVIURETERIC JUNCTION OBSTRUCTION

4 5 6

Fig No-4 & 5- USG of right kidney shows that increased pelvicaliceal system due to pelviureteric junction obstruction Fig No-6- USG shows that dilated pelvicaliceal system with intervening parenchyma suggestive of crossed fused kidney with pelviureteric junction obstruction

7 8 9 FIG NO -7- LOIN MASS DUE TO LEFT CROSSED FUSED LEFT KIDNEY WITH HYDRONEPHROSIS FIG –NO- 8&9 - INTRAVENOUS PYELOGRAPHY OF SOLITARY RIGHT KIDEY WITH HYDRONEPROSIS SHOWS DILATED PELVICALICEAL SYSTEM AND URETER NOT SEEN ON RIGHT SIDE.ON LEFT SIDE THERE IS NO CONTRAST SEEN

9 11 12 FIG NO – 10- computed tomography with contrast shows solitary right kidney with hydronephrosis with thin renal parenchyma and absent left kidney FIG NO – 11- Abdominal CT with contrast of a patient with PUJ obstruction demonstrates massive dilation of the right renal pelvis and calyces, with thinning of the right renal cortex. The left kidney is normal in size and appearance. FIG NO – 12-CT Scan of the left pelvic kidney with hydronephrosis

INTRAVENOUS PYELOGRAPHY WITH CONTRAST

13 14 15 FIG NO- 13 - INTRAVENOUS PYELOGRAPHY OF ANTENATALLY DIAGNOSED CHILD SHOWS FAINT NEPHROGRAPHIC EFFECT ON RIGHT SIDE DUE TO RITHT PUJ OBSTRUCTION FIG NO- 14 - INTRAVENOUS PYELOGRAPHY SHOWS LEFT HYDRONEPHROSIS WITH RIM SIGN. FIG NO-15 – INTRAVENOUS PYELOGRAPHY SHOWS RIGHT SIDE GRADE III HYDRONEPHROSIS WITH NO URETER SEEN. – RIGHT PELVIURETERIC OBSTRUCTION,LEFT KIDNEY NORMAL

16 17 18 FIG NO - 16 - INTRAVENOUS PYELOGRAPHY SHOWS LEFT PELVIC KIDNEY WITH HYDRONEPHROSIS FIG NO -17&18 - MR UROGRPHY SHOWS BILATERAL PELVICALICEAL SYSTEM DILATATION DUE TO BILATERAL PELVIURETERIC OBSTRUCTION

Fig No-19- Pre operative diuretic renogram Fig No-20- Post operative diuretic renogram

Preperative Diuretic renal scan shows normal uptake and excretion of radiotracer from the right kidney into the right ureter and bladder. The progressive uptake of contrast material into the left renal collecting system without excretion is consistent with pelviureteric junction obstruction Postoperative follow up renogram demonstrates renal tracer accumulation and excretion of left kidney. The normal right kidney demonstrates prompt uptake and excretion.

FIG NO-21- OPERATIVE STEPS OF ANDERSON HYNES PYELOPLASTY

1 2 3

4 5 6

7 Fig No-1and 2 - Through anterolateral incision left kidney approached extraperitoneally Fig No- 3-Dilated Pelvis and ureter were identified and narrowed PUJ was dismembered. Fig No-4, 5 &6- Ureter was spatulated and dependent site anastomosis completed. Fig no-7- wound closed in layers with drainage tube.

FIG NO-22-POST OPERATIVE FOLLOW UP INTRAVENOUS PYELOGRAM

1 2

3 4 Intravenous pyelogram shows

1. Preoperative film shows left sided hydronephrosis with no ureter seen 2. Postoperative 1 hour picture shows dye in the left pelvicaliceal system. 3. Post op 3 hour picture shows the dye is completely drained from the left pelvis 4. post pyeloplasty USG shows dilated pelvicaliceal system right kidney